AAAS

Summary

Gene Symbol: AAAS
Description: aladin WD repeat nucleoporin
Alias: AAA, AAASb, ADRACALA, ADRACALIN, ALADIN, GL003, aladin, Allgrove, triple-A, achalasia, adrenocortical insufficiency, alacrimia
Species: human
Products:     AAAS

Top Publications

  1. Sandrini F, Farmakidis C, Kirschner L, Wu S, Tullio Pelet A, Lyonnet S, et al. Spectrum of mutations of the AAAS gene in Allgrove syndrome: lack of mutations in six kindreds with isolated resistance to corticotropin. J Clin Endocrinol Metab. 2001;86:5433-7 pubmed
    ..AAAS codes for the WD-repeat containing ALADIN (for alacrima-achalasia-adrenal insufficiency-neurologic disorder) protein...
  2. Tullio Pelet A, Salomon R, Hadj Rabia S, Mugnier C, De Laet M, Chaouachi B, et al. Mutant WD-repeat protein in triple-A syndrome. Nat Genet. 2000;26:332-5 pubmed
    ..The predicted product of AAAS, ALADIN (for alacrima-achalasia-adrenal insufficiency neurologic disorder), belongs to the WD-repeat family of regulatory ..
  3. Handschug K, Sperling S, Yoon S, Hennig S, Clark A, Huebner A. Triple A syndrome is caused by mutations in AAAS, a new WD-repeat protein gene. Hum Mol Genet. 2001;10:283-90 pubmed
    ..affected in triple A syndrome, supporting the hypothesis that mutations in this triple A syndrome gene (AAAS) are responsible for the disease...
  4. Schmittmann Ohters K, Huebner A, Richter Unruh A, Hauffa B. Clinical and novel molecular findings in a 6.8-year-old Turkish boy with triple A syndrome. Horm Res. 2001;56:67-72 pubmed
    ..Further, a homozygous G -->A transition in exon 9 of the newly identified AAAS gene, resulting in a stop codon (W295X) and predicting a truncated protein with loss of function, confirmed the ..
  5. Goizet C, Catargi B, Tison F, Tullio Pelet A, Hadj Rabia S, Pujol F, et al. Progressive bulbospinal amyotrophy in triple A syndrome with AAAS gene mutation. Neurology. 2002;58:962-5 pubmed
    ..The gene responsible, AAAS, recently has been identified...
  6. Cho A, Yang K, Bae Y, Bahk Y, Kim E, Lee H, et al. Tissue-specific expression and subcellular localization of ALADIN, the absence of which causes human triple A syndrome. Exp Mol Med. 2009;41:381-6 pubmed publisher
    ..aspartic acid (WD) repeat-containing protein named alacrima-achalasia-adrenal insufficiency neurologic disorder (ALADIN). Northern blot analysis shows that the 2...
  7. Huebner A, Kaindl A, Knobeloch K, Petzold H, Mann P, Koehler K. The triple A syndrome is due to mutations in ALADIN, a novel member of the nuclear pore complex. Endocr Res. 2004;30:891-9 pubmed
    ..The triple A syndrome is caused by mutations in the AAAS gene, which encodes a protein known as ALADIN (ALacrima Achalasia aDrenal Insufficiency Neurologic disorder)...
  8. Cronshaw J, Krutchinsky A, Zhang W, Chait B, Matunis M. Proteomic analysis of the mammalian nuclear pore complex. J Cell Biol. 2002;158:915-27 pubmed
    ..One of these WD repeat nucleoporins is ALADIN, the gene mutated in triple-A (or Allgrove) syndrome...
  9. Prpic I, Huebner A, Persic M, Handschug K, Pavletic M. Triple A syndrome: genotype-phenotype assessment. Clin Genet. 2003;63:415-7 pubmed
    ..Associated features of the syndrome are neurological and dermatological abnormalities. Until the discovery of the AAAS gene as the responsible gene in triple A syndrome, the diagnosis was based on characteristic clinical features...

More Information

Publications127 found, 100 shown here

  1. Roubergue A, Apartis E, Vidailhet M, Mignot C, Tullio Pelet A, Lyonnet S, et al. Myoclonus and generalized digestive dysmotility in triple A syndrome with AAAS gene mutation. Mov Disord. 2004;19:344-6 pubmed
    ..These features, not previously described, illustrate an extension of the cerebral and digestive neurological involvement in this syndrome. ..
  2. Brooks B, Kleta R, Caruso R, Stuart C, Ludlow J, Stratakis C. Triple-A syndrome with prominent ophthalmic features and a novel mutation in the AAAS gene: a case report. BMC Ophthalmol. 2004;4:7 pubmed
    ..Mutations have been found in the AAAS gene on 12q13...
  3. Krumbholz M, Koehler K, Huebner A. Cellular localization of 17 natural mutant variants of ALADIN protein in triple A syndrome - shedding light on an unexpected splice mutation. Biochem Cell Biol. 2006;84:243-9 pubmed
    ..Mutations in the AAAS gene on chromosome 12q13 are responsible for the disorder. AAAS encodes a protein named ALADIN, which belongs to the family of WD-repeat-containing proteins and has been shown to localize to nuclear pore ..
  4. Milenkovic T, Zdravkovic D, Savic N, Todorovic S, Mitrovic K, Koehler K, et al. Triple A syndrome: 32 years experience of a single centre (1977-2008). Eur J Pediatr. 2010;169:1323-8 pubmed publisher
    ..Mutations in the AAAS gene on chromosome 12q13 encoding the nuclear pore protein ALADIN have been reported in these patients...
  5. Cronshaw J, Matunis M. The nuclear pore complex protein ALADIN is mislocalized in triple A syndrome. Proc Natl Acad Sci U S A. 2003;100:5823-7 pubmed
    ..Triple A syndrome arises from mutations in a WD-repeat protein of unknown function called ALADIN (also termed Adracalin or AAAS)...
  6. Storr H, Clark A, Priestley J, Michael G. Identification of the sites of expression of triple A syndrome mRNA in the rat using in situ hybridisation. Neuroscience. 2005;131:113-23 pubmed
    ..to as the triple A syndrome gene (AAAS) and which has recently been shown to encode a nuclear pore protein named ALADIN (alacrima, achalasia, adrenal insufficiency neurologic disorder)...
  7. Storr H, Kind B, Parfitt D, Chapple J, Lorenz M, Koehler K, et al. Deficiency of ferritin heavy-chain nuclear import in triple a syndrome implies nuclear oxidative damage as the primary disease mechanism. Mol Endocrinol. 2009;23:2086-94 pubmed publisher
    ..a novel, 60-kDa WD-repeat nuclear pore protein, alacrima-achalasia-adrenal insufficiency neurological disorder (ALADIN) of unknown function...
  8. Jühlen R, Peitzsch M, Gärtner S, Landgraf D, Eisenhofer G, Huebner A, et al. Compensation for chronic oxidative stress in ALADIN null mice. Biol Open. 2018;7: pubmed publisher
    Mutations in the AAAS gene coding for the nuclear pore complex protein ALADIN lead to the autosomal recessive disorder triple A syndrome...
  9. Dumic M, Putarek N, Kusec V, Barisic N, Koehler K, Huebner A. Low bone mineral density for age/osteoporosis in triple A syndrome-an overlooked symptom of unexplained etiology. Osteoporos Int. 2016;27:521-6 pubmed publisher
    ..mutations in the AAAS gene which encodes the protein alacrima achalasia adrenal insufficiency neurologic disorder (ALADIN)...
  10. Martorell S, Hueso L, Gonzalez Navarro H, Collado A, Sanz M, Piqueras L. Vitamin D Receptor Activation Reduces Angiotensin-II-Induced Dissecting Abdominal Aortic Aneurysm in Apolipoprotein E-Knockout Mice. Arterioscler Thromb Vasc Biol. 2016;36:1587-97 pubmed publisher
    Abdominal aortic aneurysm (AAA) is a vascular disorder characterized by chronic inflammation of the aortic wall...
  11. Menges A, Busch A, Reutersberg B, Trenner M, Kath P, Chernogubova E, et al. The structural atrophy of the aneurysm wall in secondary expanding aortic aneurysms with endoleak type II. J Vasc Surg. 2019;: pubmed publisher
    ..Control tissues included 42 AAAs and 13 control aortae published previously...
  12. Vangompel M, Nguyen K, Hall D, Dauer W, Rose L. A novel function for the Caenorhabditis elegans torsin OOC-5 in nucleoporin localization and nuclear import. Mol Biol Cell. 2015;26:1752-63 pubmed publisher
    Torsin proteins are AAA+ ATPases that localize to the endoplasmic reticular/nuclear envelope (ER/NE) lumen. A mutation that markedly impairs torsinA function causes the CNS disorder DYT1 dystonia...
  13. Kigawa Y, Miyazaki T, Lei X, Nakamachi T, Oguchi T, Kim Kaneyama J, et al. NADPH oxidase deficiency exacerbates angiotensin II-induced abdominal aortic aneurysms in mice. Arterioscler Thromb Vasc Biol. 2014;34:2413-20 pubmed publisher
    ..We therefore assessed the role of NOX2 in an experimental model of abdominal aortic aneurysm (AAA) and assessed the mechanism of NOX2 action in AAA...
  14. Holecek M. Ammonia and amino acid profiles in liver cirrhosis: effects of variables leading to hepatic encephalopathy. Nutrition. 2015;31:14-20 pubmed publisher
    ..Aromatic amino acids (AAA; phenylalanine, tyrosine, and tryptophan) and methionine increase due to portosystemic shunts and reduced ability ..
  15. Qin Y, Wang Y, Liu O, Jia L, Fang W, Du J, et al. Tauroursodeoxycholic Acid Attenuates Angiotensin II Induced Abdominal Aortic Aneurysm Formation in Apolipoprotein E-deficient Mice by Inhibiting Endoplasmic Reticulum Stress. Eur J Vasc Endovasc Surg. 2017;53:337-345 pubmed publisher
    Abdominal aortic aneurysm (AAA) is characterised by the infiltration of smooth muscle cell (SMC) apoptosis, inflammatory cells, neovascularisation, and degradation of the extracellular matrix...
  16. Vasiliadou I, Sánchez Vázquez R, Molina R, Martinez F, Melero J, Bautista L, et al. Biological removal of pharmaceutical compounds using white-rot fungi with concomitant FAME production of the residual biomass. J Environ Manage. 2016;180:228-37 pubmed publisher
    ..Lower removals were achieved for 4-acetamidoantipyrin (AAA), clofibric acid (ACF), atenolol (ATN), caffeine (CFN), carbamazepine (CZP), hydrochlorothiazide (HCT), ..
  17. Ntzouvani A, Nomikos T, Panagiotakos D, Fragopoulou E, Pitsavos C, McCann A, et al. Amino acid profile and metabolic syndrome in a male Mediterranean population: A cross-sectional study. Nutr Metab Cardiovasc Dis. 2017;27:1021-1030 pubmed publisher
    ..The branched-chain amino acids/aromatic amino acids (BCAA/AAA) related pattern and the glutamine/glycine/serine/asparagine (Gln/Gly/Ser/Asn) related pattern were significantly ..
  18. Acar Y, Tezel O, Salman N, Cevik E, Algaba Montes M, Oviedo García A, et al. 12th WINFOCUS world congress on ultrasound in emergency and critical care. Crit Ultrasound J. 2016;8:12 pubmed publisher
    ..hospitalEdina Ćatić Ćuti, Stanko Belina, Tihomir Vančina, Idriz KovačevićA15 The challenge of AAA: unusual case of obstructive jaundiceEdina Ćatić Ćuti, Nadan RustemovićA16 Educational ..
  19. Nakao T, Horie T, Baba O, Nishiga M, Nishino T, Izuhara M, et al. Genetic Ablation of MicroRNA-33 Attenuates Inflammation and Abdominal Aortic Aneurysm Formation via Several Anti-Inflammatory Pathways. Arterioscler Thromb Vasc Biol. 2017;37:2161-2170 pubmed publisher
    Abdominal aortic aneurysm (AAA) is an increasingly prevalent and ultimately fatal disease with no effective pharmacological treatment...
  20. Jia W, Wang J, Xu H, Li G. Resistance of Stenotrophomonas maltophilia to Fluoroquinolones: Prevalence in a University Hospital and Possible Mechanisms. Int J Environ Res Public Health. 2015;12:5177-95 pubmed publisher
    ..maltophilia isolates, while smqnr gene was found in 25.4% of total isolates. Glu-Lys mutation (GAA-AAA) was detected at the 151th amino acid of the gyrA gene, while Gly-Arg mutation (GGC-CGC) was found at the 37th ..
  21. Wang Q, Ding Y, Song P, Zhu H, Okon I, Ding Y, et al. Tryptophan-Derived 3-Hydroxyanthranilic Acid Contributes to Angiotensin II-Induced Abdominal Aortic Aneurysm Formation in Mice In Vivo. Circulation. 2017;136:2271-2283 pubmed publisher
    ..However, the causative link between dysregulated tryptophan metabolism and abdominal aortic aneurysm (AAA) is unknown...
  22. Zhou T, Wang Q, Phan N, Ren J, Yang H, Feldman C, et al. Identification of a novel class of RIP1/RIP3 dual inhibitors that impede cell death and inflammation in mouse abdominal aortic aneurysm models. Cell Death Dis. 2019;10:226 pubmed publisher
    ..gene deletion of Rip3 or pharmacological inhibition of RIP1 attenuated pathogenesis of abdominal aortic aneurysm (AAA), a life-threatening degenerative vascular disease characterized by depletion of smooth muscle cells (SMCs), ..
  23. Bath J, Dombrovskiy V, Vogel T. Impact of Patient Safety Indicators on readmission after abdominal aortic surgery. J Vasc Nurs. 2018;36:189-195 pubmed publisher
    ..PSI) events after open surgical repair (OSR) or endovascular aneurysm repair (EVAR) of abdominal aortic aneurysm (AAA) were associated with increased risk of readmission...
  24. Ghoshal K, Datta J, Majumder S, Bai S, Kutay H, Motiwala T, et al. 5-Aza-deoxycytidine induces selective degradation of DNA methyltransferase 1 by a proteasomal pathway that requires the KEN box, bromo-adjacent homology domain, and nuclear localization signal. Mol Cell Biol. 2005;25:4727-41 pubmed
    ..Mutation of the conserved KEN box, a targeting signal for proteasomal degradation, to AAA increased the basal level of Dnmt1 and blocked its degradation by 5-aza-CdR...
  25. Miao X, Siu K, Cai H. Nifedipine attenuation of abdominal aortic aneurysm in hypertensive and non-hypertensive mice: Mechanisms and implications. J Mol Cell Cardiol. 2015;87:152-9 pubmed publisher
    Rupture of abdominal aortic aneurysm (AAA) is a lethal event. No oral medicine has been available to prevent or treat AAA...
  26. Wang S, Green L, Gutwein A, Gupta A, Babbey C, Motaganahalli R, et al. Osteopontin may be a driver of abdominal aortic aneurysm formation. J Vasc Surg. 2018;68:22S-29S pubmed publisher
    ..inflammatory extracellular matrix protein, is involved in the formation and growth of abdominal aortic aneurysms (AAAs). However, the mechanism by which this occurs continues to be nebulous...
  27. Maegdefessel L, Azuma J, Toh R, Deng A, Merk D, Raiesdana A, et al. MicroRNA-21 blocks abdominal aortic aneurysm development and nicotine-augmented expansion. Sci Transl Med. 2012;4:122ra22 pubmed publisher
    Identification and treatment of abdominal aortic aneurysm (AAA) remains among the most prominent challenges in vascular medicine...
  28. van t Hof F, Vaucher J, Holmes M, de Wilde A, Baas A, Blankensteijn J, et al. Genetic variants associated with type 2 diabetes and adiposity and risk of intracranial and abdominal aortic aneurysms. Eur J Hum Genet. 2017;25:758-762 pubmed publisher
    ..that type 2 diabetes (T2D) is inversely associated with intracranial aneurysms (IA) and abdominal aortic aneurysms (AAA)...
  29. Ascoli Marchetti A, Pratesi G, Di Giulio L, Battistini M, Massoud R, Ippoliti A. EVAR and OPEN treatment of abdominal aortic aneurysm: What is the role of MMP-9 in the follow-up?. J Med Vasc. 2017;42:21-28 pubmed publisher
    ..role of metalloproteinases in endovascular repair (EVAR) and OPEN surgery treatment for abdominal aortic aneurysm (AAA)...
  30. Khashram M, Jones G, Roake J. Prevalence of abdominal aortic aneurysm (AAA) in a population undergoing computed tomography colonography in Canterbury, New Zealand. Eur J Vasc Endovasc Surg. 2015;50:199-205 pubmed publisher
    There is compelling level 1 evidence in support of screening men for abdominal aortic aneurysm (AAA) to reduce AAA mortality. However, New Zealand (NZ) lacks data on AAA prevalence, and national screening has not been implemented...
  31. Gallitto E, Gargiulo M, Mascoli C, Freyrie A, de Matteis M, Serra C, et al. Persistent type II endoleak after EVAR: the predictive value of the AAA thrombus volume. J Cardiovasc Surg (Torino). 2015;: pubmed
    ..of ELIIp, their pre-operative morphological predictive features (PMF) and the post-EVAR abdominal aortic aneurysm (AAA) evolution in the presence of ELIIp. Patients underwent EVAR between 2008 and 2010 were prospectively collected...
  32. Imai H, Shima T, Sutoh K, Walker M, Knight P, Kon T, et al. Direct observation shows superposition and large scale flexibility within cytoplasmic dynein motors moving along microtubules. Nat Commun. 2015;6:8179 pubmed publisher
    Cytoplasmic dynein is a dimeric AAA(+) motor protein that performs critical roles in eukaryotic cells by moving along microtubules using ATP...
  33. Timaran D, Knowles M, Soto Gonzalez M, Modrall J, TSAI S, Kirkwood M, et al. Gender and perioperative outcomes after fenestrated endovascular repair using custom-made and off-the-shelf devices. J Vasc Surg. 2016;64:267-272 pubmed publisher
    ..During a 2-year period, 79 patients (63 men [80%] and 16 women [20%]) underwent FEVAR using Zenith Fenestrated AAA Endovascular Grafts, investigational Zenith pivot branch (p-branch) devices and fenestrated custom-made devices...
  34. Berge C, Hagen A, Myhre H, Dahl T. Preoperative White Blood Cell Count in Patients with Abdominal Aortic Aneurysms and Its Relation to Survival following Surgery. Ann Vasc Surg. 2017;41:127-134 pubmed publisher
    ..WBC) count may predict 30-day mortality and long-term survival following surgery for abdominal aortic aneurysm (AAA). Secondarily, we wanted to assess the potential sex differences in WBC in these patients...
  35. Steely A, Callas P, NEAL D, Scali S, Goodney P, Schanzer A, et al. Regional Variation in Postoperative Myocardial Infarction in Patients Undergoing Vascular Surgery in the United States. Ann Vasc Surg. 2017;40:63-73 pubmed publisher
    ..were included in the analysis: carotid endarterectomy (CEA, n = 39,118), endovascular abdominal aortic aneurysm (AAA) repair (EVAR, n = 15,106), infrainguinal bypass (INFRA, n = 17,176), and open infrarenal AAA repair (OAAA, n =..
  36. Tin K, Sobani Z, Horovitz J, Rahmani R. Aortic and splanchnic artery aneurysms: Unusual causes of biliary obstruction - A retrospective cohort from literature. Int J Surg. 2017;39:163-168 pubmed publisher
    ..39 cases of biliary obstruction secondary to acquired aortic or splanchnic vessel abnormalities; 16 were caused by AAAs and 23 by splanchnic vessels...
  37. Wohlever M, Mateja A, McGilvray P, Day K, Keenan R. Msp1 Is a Membrane Protein Dislocase for Tail-Anchored Proteins. Mol Cell. 2017;67:194-202.e6 pubmed publisher
    ..Msp1 is a transmembrane AAA-ATPase, but its role in TA protein clearance is not known...
  38. Hanvold S, Vinknes K, Bastani N, Turner C, Løken E, Mala T, et al. Plasma amino acids, adiposity, and weight change after gastric bypass surgery: are amino acids associated with weight regain?. Eur J Nutr. 2018;57:2629-2637 pubmed publisher
    ..In a prospective study with 165 patients, we examined the relationship between plasma aromatic AAs (AAAs), branched chain AAs (BCAAs), and total cysteine (tCys) 2 years after RYGB, with BMI at 2 years and with weight ..
  39. Steczko J, Bax K, Ash S. Effect of hemodiabsorption and sorbent-based pheresis on amino acid levels in hepatic failure. Int J Artif Organs. 2000;23:375-88 pubmed
    ..and the PF-Unit have the capability to selectively remove various amino acids, especially aromatic amino acids (AAA)...
  40. Martin Gonzalez T, Pinçon C, Hertault A, Maurel B, Labbé D, Spear R, et al. Renal outcomes analysis after endovascular and open aortic aneurysm repair. J Vasc Surg. 2015;62:569-77 pubmed publisher
    ..and renal volume) after endovascular aneurysm repair (EVAR) and open repair (OR) of abdominal aortic aneurysm (AAA). All AAA repairs performed between November 2009 and July 2011 were included in this retrospective study...
  41. Lo J, Jedynak J, Chuen J. Perceived barriers to endovascular repair of ruptured abdominal aortic aneurysm among Australasian vascular surgeons. J Vasc Surg. 2016;64:328-332 pubmed publisher
    Although endovascular abdominal aortic aneurysm (AAA) repair (EVAR) is widely accepted for elective surgery, the uptake of emergency EVAR for ruptured AAA (REVAR) has trailed behind...
  42. Samoila G, Ford R, Glasbey J, Lewis M, Twine C, Williams I. The Significance of Hypothermia in Abdominal Aortic Aneurysm Repair. Ann Vasc Surg. 2017;38:323-331 pubmed publisher
    ..In ruptured AAAs, the lowest average intraoperative temperature was recorded in open repair compared with EVAR (P = 0.02)...
  43. Hölscher D, Buerkert A, Schneider B. Phenylphenalenones Accumulate in Plant Tissues of Two Banana Cultivars in Response to Herbivory by the Banana Weevil and Banana Stem Weevil. Plants (Basel). 2016;5: pubmed publisher
    ..Grande Naine" (AAA) and Musa acuminata × balbisiana Colla cv...
  44. Sysoeva T. Assessing heterogeneity in oligomeric AAA+ machines. Cell Mol Life Sci. 2017;74:1001-1018 pubmed publisher
    ATPases Associated with various cellular Activities (AAA+ ATPases) are molecular motors that use the energy of ATP binding and hydrolysis to remodel their target macromolecules...
  45. Khashram M, Pitama S, Williman J, Jones G, Roake J. Survival Disparity Following Abdominal Aortic Aneurysm Repair Highlights Inequality in Ethnic and Socio-economic Status. Eur J Vasc Endovasc Surg. 2017;54:689-696 pubmed publisher
    ..of this study was to investigate how SES and ethnicity impacted patient survival after abdominal aortic aneurysm (AAA) repair. Consecutive patients undergoing open and endovascular AAA repair during a 14.5 year period were identified...
  46. Thurnher S, Cejna M. Imaging of aortic stent-grafts and endoleaks. Radiol Clin North Am. 2002;40:799-833 pubmed
    ..High-quality ultrasound reliably excludes endoleaks in patients after stent-grafting of AAAs. There is a substantial variability, however, in measuring the diameter of aneurysm sacs; thus, confirmation using ..
  47. Mazzaccaro D, Occhiuto M, Stegher S, Righini P, Malacrida G, Nano G. Tips About the Cordis INCRAFT Endograft. Ann Vasc Surg. 2016;30:205-10 pubmed publisher
    ..has been introduced in the clinical practice of endovascular aortic repair (EVAR) for the treatment of infrarenal AAAs (iAAAs). In our operative unit, it has been used since November 2014...
  48. Do T, Shih P, Chang C, Lin C, Lin M. Rich magneto-absorption spectra of AAB-stacked trilayer graphene. Phys Chem Chem Phys. 2016;18:17597-605 pubmed publisher
    ..There exist extra single peaks because of LL anti-crossings. AAB, AAA, ABA, and ABC stackings considerably differ from one another in terms of the inter-LL category, frequency, ..
  49. Takagi H. Association of diabetes mellitus with presence, expansion, and rupture of abdominal aortic aneurysm: "Curiouser and curiouser!" cried ALICE. Semin Vasc Surg. 2016;29:18-26 pubmed publisher
    ..representative atherosclerotic diseases that are positively associated with presence of abdominal aortic aneurysm (AAA)...
  50. Mazzaccaro D, Nano G, Settembrini A, Carmo M, Dallatana R, Salvati S, et al. Open and endovascular elective treatment of abdominal aortic aneurysms: a real-world experience. Surg Today. 2017;47:1347-1355 pubmed publisher
    To present a real-world experience of the elective treatment of abdominal aortic aneurysms (AAAs) using both open repair (OR) and endovascular repair (EVAR)...
  51. Dubois L, Allen B, Bray Jenkyn K, Power A, DeRose G, Forbes T, et al. Higher surgeon annual volume, but not years of experience, is associated with reduced rates of postoperative complications and reoperations after open abdominal aortic aneurysm repair. J Vasc Surg. 2018;67:1717-1726.e5 pubmed publisher
    Volume-outcome relationships for open abdominal aortic aneurysm (AAA) repair have received less attention in publicly funded health systems...
  52. Phillips E, Yrineo A, Schroeder H, Wilson K, Cheng J, Goergen C. Morphological and Biomechanical Differences in the Elastase and AngII apoE(-/-) Rodent Models of Abdominal Aortic Aneurysms. Biomed Res Int. 2015;2015:413189 pubmed publisher
    ..While each model develops AAAs specific to their induction method, they both share characteristics with human aneurysms, such as marked changes in ..
  53. Anderson D, Le Moigne R, Djakovic S, Kumar B, Rice J, Wong S, et al. Targeting the AAA ATPase p97 as an Approach to Treat Cancer through Disruption of Protein Homeostasis. Cancer Cell. 2015;28:653-665 pubmed publisher
    p97 is a AAA-ATPase with multiple cellular functions, one of which is critical regulation of protein homeostasis pathways. We describe the characterization of CB-5083, a potent, selective, and orally bioavailable inhibitor of p97...
  54. Chiu H, Chu T, Cheng Y, Tsai T. The Association between Clinical Response to Ustekinumab and Immunogenicity to Ustekinumab and Prior Adalimumab. PLoS ONE. 2015;10:e0142930 pubmed publisher
    ..was comparable between patients who had failed previous adalimumab with or without anti-adalimumab antibodies (AAA) (14.3% vs. 12.5%, p = 1.00). However, a higher proportion of switchers without AAA obtaining PASI50 (71.4% vs. 37...
  55. Broos P, t Mannetje Y, Stokmans R, Houterman S, Corte G, Cuypers P, et al. A 15-Year Single-Center Experience of Endovascular Repair for Elective and Ruptured Abdominal Aortic Aneurysms. J Endovasc Ther. 2016;23:566-73 pubmed publisher
    ..6%) patients (mean age 72 years; 697 men) with asymptomatic or symptomatic abdominal aortic aneurysms (AAAs) were assigned to the el-EVAR group; 90 (10...
  56. Arnaoutoglou E, Kouvelos G, Tzimas P, Laou E, Bouris V, Papadopoulos G, et al. Relationship between normal preoperative white blood cell count and major adverse events after endovascular repair for abdominal aortic aneurysm: results of a pilot study. J Clin Anesth. 2017;36:201-205 pubmed publisher
    ..the reference range and major adverse events (MAEs) following endovascular repair of abdominal aortic aneurysms (AAA). Prospective observational study. Vascular surgery clinic in a tertiary university hospital...
  57. Xuan H, Xu B, Wang W, Tanaka H, Fujimura N, Miyata M, et al. Inhibition or deletion of angiotensin II type 1 receptor suppresses elastase-induced experimental abdominal aortic aneurysms. J Vasc Surg. 2018;67:573-584.e2 pubmed publisher
    ..receptor (AT1) activation is essential for the development of exogenous Ang II-induced abdominal aortic aneurysms (AAAs) in hyperlipidemic animals...
  58. Bedmutha A, Singh N, Shivdasani D. Metabolic Imaging as a Novel Strategy in Evaluation of Mycotic Abdominal Aortic Aneurysm: A Case Report and Brief Clinical Review. Indian J Nucl Med. 2017;32:336-339 pubmed publisher
    Abdominal aortic aneurysm (AAA) is an uncommon entity with high mortality. Etiologically, they are classified as inflammatory and infective (mycotic), the latter being less common...
  59. Wang Y, Joannic D, Juillion P, Monnet A, Delassus P, Lalande A, et al. Validation of the strain assessment of an abdominal phantom aortic aneurysm: comparison of results obtained from magnetic resonance imaging measurements and stereovision measurements. J Biomech Eng. 2017;: pubmed publisher
    ..paper aims to demonstrate the feasibility of this measure by MRI on phantom realistic abdominal aortic aneurysm (AAA).The aortic geometry is obtained from series of cine-MR images and reconstructed using Mimics® software...
  60. Miniowitz Shemtov S, Eytan E, Kaisari S, Sitry Shevah D, Hershko A. Mode of interaction of TRIP13 AAA-ATPase with the Mad2-binding protein p31comet and with mitotic checkpoint complexes. Proc Natl Acad Sci U S A. 2015;112:11536-40 pubmed publisher
    The AAA-ATPase thyroid hormone receptor interacting protein 13 (TRIP13), jointly with the Mad2-binding protein p31(comet), promotes the inactivation of the mitotic (spindle assembly) checkpoint by disassembling the mitotic checkpoint ..
  61. Chun K, Schmidt A, Bains S, Nguyen A, Samadzadeh K, Wilson M, et al. Surveillance outcomes of small abdominal aortic aneurysms identified from a large screening program. J Vasc Surg. 2016;63:55-61 pubmed publisher
    ..Therefore, physician ordering of scans may exert the greatest influence on follow-up rates in patients with small AAA. Automatic ordering of follow-up scans for patients with small AAAs is proposed to improve follow-up rates.
  62. Hynes C, Endicott K, Iranmanesh S, Amdur R, Macsata R. Reoperation rates after open and endovascular abdominal aortic aneurysm repairs. J Vasc Surg. 2017;65:1323-1328 pubmed publisher
    This study compared reoperation rates associated with open abdominal aortic aneurysm (AAA) repair (OR) outcomes vs endovascular AAA repair (EVAR)...
  63. Floros N, Papadakis M, Wagenhäuser M, Duran M, Simon F, Schelzig H, et al. Outcomes after Open Surgery for Inflammatory Abdominal Aortic Aneurysm: A 10-Year Single-Center Experience. Ann Vasc Surg. 2017;43:144-150 pubmed publisher
    ..We retrospectively reviewed the medical records of 18 patients with IAAA selected out of patients with AAA who underwent open surgery in the Department of Vascular and Endovascular Surgery at the University Hospital ..
  64. Michel M, Becquemin J, Marzelle J, Quelen C, Durand Zaleski I. Editor's Choice - A Study of the Cost-effectiveness of Fenestrated/branched EVAR Compared with Open Surgery for Patients with Complex Aortic Aneurysms at 2 Years. Eur J Vasc Endovasc Surg. 2018;56:15-21 pubmed publisher
    ..EVAR) compared with open surgical repair (OSR) in thoraco-abdominal or complex abdominal aortic aneurysms (TAAA/AAA) at 2 years. Two matched cohorts of patients with TAAA or complex AAA were compared after a follow-up of two years...
  65. Blok N, Tan D, Wang R, Penczek P, Baker D, DiMaio F, et al. Unique double-ring structure of the peroxisomal Pex1/Pex6 ATPase complex revealed by cryo-electron microscopy. Proc Natl Acad Sci U S A. 2015;112:E4017-25 pubmed publisher
    Members of the AAA family of ATPases assemble into hexameric double rings and perform vital functions, yet their molecular mechanisms remain poorly understood...
  66. Orion K, Beaulieu R, Black J. Aortocaval Fistula: Is Endovascular Repair the Preferred Solution?. Ann Vasc Surg. 2016;31:221-8 pubmed publisher
    ..of open and endovascular repair of aortocaval fistulas (ACFs) in the setting of abdominal aortic aneurysms (AAAs)...
  67. Harel T, Yoon W, Garone C, Gu S, Coban Akdemir Z, Eldomery M, et al. Recurrent De Novo and Biallelic Variation of ATAD3A, Encoding a Mitochondrial Membrane Protein, Results in Distinct Neurological Syndromes. Am J Hum Genet. 2016;99:831-845 pubmed publisher
    ATPase family AAA-domain containing protein 3A (ATAD3A) is a nuclear-encoded mitochondrial membrane protein implicated in mitochondrial dynamics, nucleoid organization, protein translation, cell growth, and cholesterol metabolism...
  68. Hou Y, Chaudhary S, Gao F, Li Z. Surgical follow-up results for apocrine adenosis and atypical apocrine adenosis diagnosed on breast core biopsy. Ann Diagn Pathol. 2016;24:4-6 pubmed publisher
    Apocrine adenosis (AA) and atypical apocrine adenosis (AAA) are uncommon findings in breast biopsies that may be misinterpreted as carcinoma...
  69. Reddy G, Mukhopadhyay A, Dey C. Characterization of ciliobrevin A mediated dynein ATPase inhibition on flagellar motility of Leishmania donovani. Mol Biochem Parasitol. 2017;214:75-81 pubmed publisher
    Axonemal dyneins are members of AAA+ proteins involved in force generation and are responsible for flagellar motility in eukaryotes...
  70. Lanjuan L, Qian Y, Jianrong H, Xiaowei X, Yuemei C, Yagang C, et al. Severe hepatitis treated with an artificial liver support system. Int J Artif Organs. 2001;24:297-303 pubmed
    ..and HBV-DNA concentrations were significantly decreased, and the serum concentration of aromatic amino acids (AAA) such as methionine decreased while BCAA/AAA ratio increased...
  71. Gallitto E, Gargiulo M, Freyrie A, Bianchini Massoni C, Pini R, Mascoli C, et al. Results of standard suprarenal fixation endografts for abdominal aortic aneurysms with neck length ?10 mm in high-risk patients unfit for open repair and fenestrated endograft. J Vasc Surg. 2016;64:563-570.e1 pubmed publisher
    ..endovascular aneurysm repair (EVAR) using a standard suprarenal fixation endograft in abdominal aortic aneurysms (AAAs) with infrarenal neck length ?10 mm (short-neck AAA [SN-AAA])...
  72. Gupta P, Brahmbhatt R, Kempe K, Stickley S, Rohrer M. Thirty-day outcomes after fenestrated endovascular repair are superior to open repair of abdominal aortic aneurysms involving visceral vessels. J Vasc Surg. 2017;66:1653-1658.e1 pubmed publisher
    ..fenestrated endovascular aortic aneurysm repair (FEVAR) of abdominal aortic aneurysms involving visceral vessels (AAA-Vs), no multi-institutional study has compared FEVAR with open surgery (OS) for AAA-Vs...
  73. Doderer S, Gäbel G, Kokje V, Northoff B, Holdt L, Hamming J, et al. Adventitial adipogenic degeneration is an unidentified contributor to aortic wall weakening in the abdominal aortic aneurysm. J Vasc Surg. 2018;67:1891-1900.e4 pubmed publisher
    ..Interestingly, strong clinical and molecular associations exist between popliteal artery aneurysms (PAAs) and AAAs; however, PAAs have an extremely low propensity to rupture...
  74. Zhang P, Zhang Y, Sun L, Sinumporn S, Yang Z, Sun B, et al. The Rice AAA-ATPase OsFIGNL1 Is Essential for Male Meiosis. Front Plant Sci. 2017;8:1639 pubmed publisher
    ..Here, we isolated and characterized the rice OsFIGNL1 (OsFidgetin-like 1) gene, encoding a conserved AAA-ATPase, and explored its function and importance in male meiosis and pollen formation...
  75. Shen Y, Russo V, Zeglinski M, Sellers S, Wu Z, Oram C, et al. Recombinant Decorin Fusion Protein Attenuates Murine Abdominal Aortic Aneurysm Formation and Rupture. Sci Rep. 2017;7:15857 pubmed publisher
    ..Adventitial DCN is reduced in abdominal aortic aneurysm (AAA) resulting in vessel wall instability thereby predisposing the vessel to rupture...
  76. Li H, Li J, Lü C, Xia Y, Xin Y, Liu H, et al. FisR activates ?54 -dependent transcription of sulfide-oxidizing genes in Cupriavidus pinatubonensis JMP134. Mol Microbiol. 2017;105:373-384 pubmed publisher
    ..genes, encoding a ?54 -dependent transcription factor (FisR) with three domains: an R domain, an AAA+ domain and a DNA-binding domain...
  77. Desikan S, Singh N, Steele S, Tran N, Quiroga E, Danaher P, et al. The Incidence of Ischemic Colitis after Repair of Ruptured Abdominal Aortic Aneurysms Is Decreasing in the Endovascular Era. Ann Vasc Surg. 2018;47:247-252 pubmed publisher
    ..In addition, we analyzed the incidence of IC before and after the implementation of a formal rupture AAA protocol (rEVAR protocol)...
  78. Akagi D, Hoshina K, Watanabe T, Conte M. Drug therapy for abdominal aortic aneurysms utilizing omega-3 unsaturated fatty acids and their derivatives. Curr Drug Targets. 2017;: pubmed publisher
    Abdominal aortic aneurysms (AAA) are life-threatening because of the potential for rupture, resulting in death. The current standard treatment for AAA is surgery, comprising laparotomic graft replacement and endovascular repair...
  79. Dong S, Hu W, Ge Y, Ojcius D, Lin X, Yan J. A leptospiral AAA+ chaperone-Ntn peptidase complex, HslUV, contributes to the intracellular survival of Leptospira interrogans in hosts and the transmission of leptospirosis. Emerg Microbes Infect. 2017;6:e105 pubmed publisher
    ..HslUV is an ATP-dependent chaperone-peptidase complex containing ATPase associated with various cellular activity (AAA+) and N-terminal nucleophile (Ntn) hydrolase superfamily domains, respectively, which hydrolyzed casein and ..
  80. Takagi H, Umemoto T. Vitamins and abdominal aortic aneurysm. Int Angiol. 2017;36:21-30 pubmed publisher
    To summarize the association of vitamins (B6, B12, C, D, and E) and abdominal aortic aneurysm (AAA), we reviewed clinical studies with a comprehensive literature research and meta-analytic estimates...
  81. Baxter B, Matsumura J, Curci J, McBride R, Blackwelder W, Liu X, et al. Non-invasive Treatment of Abdominal Aortic Aneurysm Clinical Trial (N-TA(3)CT): Design of a Phase IIb, placebo-controlled, double-blind, randomized clinical trial of doxycycline for the reduction of growth of small abdominal aortic aneurysm. Contemp Clin Trials. 2016;48:91-8 pubmed publisher
    ..bid) for inhibition of growth in the greatest transverse, orthogonal diameter of small abdominal aortic aneurysms (AAA). We will enroll 258 patients, ≥55years of age who have AAA, men: 3.5-5.0cm and women: 3.5-4...
  82. Anastasiadou C, Giannakakis S, Papapetrou A, Galyfos G, Papacharalampous G, Maltezos C. Late Rupture of a Totally Thrombosed Abdominal Aortic Aneurysm: A Case Report and Literature Review. Ann Vasc Surg. 2018;46:368.e5-368.e8 pubmed publisher
    Chronic totally thrombosed abdominal aortic aneurysms (AAAs) comprise a rare medical situation, with only a few cases reported in literature. Optimal management has been controversial, although an early risk for rupture is present...
  83. Marie P, Plissonnier D, Bravetti S, Coscas R, Rouer M, Haulon S, et al. Low baseline and subsequent higher aortic abdominal aneurysm FDG uptake are associated with poor sac shrinkage post endovascular repair. Eur J Nucl Med Mol Imaging. 2018;45:549-557 pubmed publisher
    The growth phases of medically treated abdominal aortic aneurysms (AAA) are frequently associated with an 18F-fluorodesoxyglucose positron emission tomography (FDG-PET) pattern involving low baseline and subsequent higher FDG ..
  84. Salata K, Syed M, Hussain M, Eikelboom R, de Mestral C, Verma S, et al. Renin-angiotensin system blockade does not attenuate abdominal aortic aneurysm growth, rupture rate, or perioperative mortality after elective repair. J Vasc Surg. 2018;67:629-636.e2 pubmed publisher
    ..enzyme inhibitors (ACEis) or angiotensin receptor blockers (ARBs) on human abdominal aortic aneurysm (AAA) growth, rupture, and perioperative mortality...
  85. Quicken S, Donders W, van Disseldorp E, Gashi K, Mees B, van de Vosse F, et al. Application of an Adaptive Polynomial Chaos Expansion on Computationally Expensive Three-Dimensional Cardiovascular Models for Uncertainty Quantification and Sensitivity Analysis. J Biomech Eng. 2016;138: pubmed publisher
    ..In this study, we demonstrate the agPCE by applying it to a 3D abdominal aortic aneurysm (AAA) wall mechanics model and a 3D model of flow through an arteriovenous fistula (AVF)...
  86. Shi Y, Yang C, Wang S, Li W, Li J, Wang S. Characterization of Fc gamma receptor IIb expression within abdominal aortic aneurysm. Biochem Biophys Res Commun. 2017;485:295-300 pubmed publisher
    To evaluate protein profiles and Fc gamma receptor IIb (FCGRIIB) expression in abdominal aortic aneurysm (AAA) compared to the expression in normal aortas...
  87. Dakour Aridi H, Locham S, Nejim B, Ghajar N, Alshaikh H, Malas M. Indications, risk factors, and outcomes of 30-day readmission after infrarenal abdominal aneurysm repair. J Vasc Surg. 2018;67:747-758.e7 pubmed publisher
    ..after open aortic repair (OAR) and endovascular aneurysm repair (EVAR) of infrarenal abdominal aortic aneurysms (AAAs). A retrospective analysis of the Premier Healthcare Database from 2009 to 2015 was performed...
  88. Murtola M, Ghidini A, Virta P, Stromberg R. Zinc Ion-Dependent Peptide Nucleic Acid-Based Artificial Enzyme that Cleaves RNA-Bulge Size and Sequence Dependence. Molecules. 2017;22: pubmed publisher
    ..Compared to a starting point of targets forming 3-nt AAA bulges (half-lives of ca 21-24 h), we could identify target sequences that were cleaved with half-lives three times ..
  89. Noda Y, Nakajima M, Takahashi M, Mamanee T, Hosaka K, Takagaki T, et al. The effect of five kinds of surface treatment agents on the bond strength to various ceramics with thermocycle aging. Dent Mater J. 2017;36:755-761 pubmed publisher
    ..Ceramic plates were prepared from feldspathic ceramic; AAA, lithium disilicate ceramic material; IPS e.max Press, zirconia ceramic; Lava...
  90. Lin H, Levison B, Buffa J, Huang Y, Fu X, Wang Z, et al. Myeloperoxidase-mediated protein lysine oxidation generates 2-aminoadipic acid and lysine nitrile in vivo. Free Radic Biol Med. 2017;104:20-31 pubmed publisher
    Recent studies reveal 2-aminoadipic acid (2-AAA) is both elevated in subjects at risk for diabetes and mechanistically linked to glucose homeostasis...
  91. Guey S, Kraemer M, Herve D, Ludwig T, Kossorotoff M, Bergametti F, et al. Rare RNF213 variants in the C-terminal region encompassing the RING-finger domain are associated with moyamoya angiopathy in Caucasians. Eur J Hum Genet. 2017;25:995-1003 pubmed publisher
    ..associated to the R4810K variant in the RNF213 gene that encodes for a protein containing a RING-finger and two AAA+ domains...
  92. Zacchi L, Dittmar J, Mihalevic M, Shewan A, Schulz B, Brodsky J, et al. Early-onset torsion dystonia: a novel high-throughput yeast genetic screen for factors modifying protein levels of torsinA?E. Dis Model Mech. 2017;10:1129-1140 pubmed publisher
    ..studied and validated EOTD-associated mutation, torsinA?E, is a deletion of a C-terminal glutamate residue in the AAA+ ATPase torsinA...