PCCA

Summary

Gene Symbol: PCCA
Description: propionyl-CoA carboxylase subunit alpha
Alias: propionyl-CoA carboxylase alpha chain, mitochondrial, PCCase alpha subunit, pccA complementation group, propanoyl-CoA:carbon dioxide ligase alpha subunit, propionyl CoA carboxylase, alpha polypeptide, propionyl Coenzyme A carboxylase, alpha polypeptide, propionyl-CoA carboxylase alpha subunit
Species: human
Products:     PCCA

Top Publications

  1. Jiang H, Rao K, Yee V, Kraus J. Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli. J Biol Chem. 2005;280:27719-27 pubmed
    ..PCC consists of two heterologous subunits, alpha PCC and beta PCC, which are encoded by the nuclear PCCA and PCCB genes, respectively...
  2. Desviat L, Clavero S, Perez Cerda C, Navarrete R, Ugarte M, Perez B. New splicing mutations in propionic acidemia. J Hum Genet. 2006;51:992-7 pubmed
    Propionic acidemia results from mutations in either of the two genes, PCCA or PCCB, that encode the two subunits of the propionyl-CoA carboxylase (PCC) enzyme...
  3. Quinn L, Mann K, Jones R, Bathla S, Stremitzer S, Dunne D, et al. Defining enhanced recovery after resection of peri-hilar cholangiocarcinoma. Eur J Surg Oncol. 2019;: pubmed publisher
    Enhanced recovery after surgery (ERAS) for peri-hilar cholangiocarcinoma (pCCA) has not been described in the literature...
  4. Martin Hernandez E, Quijada Fraile P, Oliveros Leal L, García Silva M, Perez Cerda C, Baro Fernández M, et al. Nutritional and Pharmacological Management during Chemotherapy in a Patient with Propionic Acidaemia and Rhabdomyosarcoma Botryoides. JIMD Rep. 2012;6:73-8 pubmed publisher
    ..The patient is a girl with neonatal onset of propionic acidaemia, homozygous for the c.2041-2924del3889 mutation in PCCA gene. At 23 months of age she was diagnosed with genitourinary embryonal rhabdomyosarcoma...
  5. Lehrke H, Heimbach J, Wu T, Jenkins S, Gores G, Rosen C, et al. Prognostic Significance of the Histologic Response of Perihilar Cholangiocarcinoma to Preoperative Neoadjuvant Chemoradiation in Liver Explants. Am J Surg Pathol. 2016;40:510-8 pubmed publisher
    Perihilar cholangiocarcinoma (pCCA) has a dismal prognosis. Protocols incorporating chemotherapy, radiotherapy, and liver transplantation (LT) have emerged as curative options for unresectable tumors with 70% 5-year survival rates...
  6. Kraus J, Spector E, Venezia S, Estes P, Chiang P, Creadon Swindell G, et al. Mutation analysis in 54 propionic acidemia patients. J Inherit Metab Dis. 2012;35:51-63 pubmed publisher
    ..from consanguineous families, the entire PCCB gene was examined by genomic DNA sequencing and in 39 individuals the PCCA gene was also studied. In three patients we found mutations in both PCC genes...
  7. Gallego Villar L, Perez Cerda C, Perez B, Abia D, Ugarte M, Richard E, et al. Functional characterization of novel genotypes and cellular oxidative stress studies in propionic acidemia. J Inherit Metab Dis. 2013;36:731-40 pubmed publisher
    ..PA is caused by mutations in either the PCCA or PCCB genes encoding the α- and β-subunits of the PCC enzyme which are assembled as an α6β6 dodecamer...
  8. Rojas J, Dayton P. In Vivo Molecular Imaging Using Low-Boiling-Point Phase-Change Contrast Agents: A Proof of Concept Study. Ultrasound Med Biol. 2019;45:177-191 pubmed publisher
    ..Data indicate that PCCA-specific imaging is more sensitive to small numbers of bound agents than conventional contrast imaging...
  9. Rivera Barahona A, Navarrete R, Garcia Rodriguez R, Richard E, Ugarte M, Perez Cerda C, et al. Identification of 34 novel mutations in propionic acidemia: Functional characterization of missense variants and phenotype associations. Mol Genet Metab. 2018;125:266-275 pubmed publisher
    Propionic acidemia (PA) is caused by mutations in the PCCA and PCCB genes, encoding α and β subunits, respectively, of the mitochondrial enzyme propionyl-CoA carboxylase (PCC)...

More Information

Publications102 found, 100 shown here

  1. Pramar Y, Mandal T, Bostanian L, Nguyen A, Miller V, Morris T, et al. Stability of Compounded Ursodiol Suspensions in PCCA Base, SuspendIt. Int J Pharm Compd. 2019;23:70-76 pubmed
    ..of this study was to determine the physicochemical stability of extemporaneously compounded ursodiol suspensions in PCCA base SuspendIt...
  2. Tang Z, Yang Y, Zhao Z, Wei K, Meng W, Li X. The clinicopathological factors associated with prognosis of patients with resectable perihilar cholangiocarcinoma: A systematic review and meta-analysis. Medicine (Baltimore). 2018;97:e11999 pubmed publisher
    ..combined with the preoperative management has improved the resectability rate of perihilar cholangiocarcinoma (pCCA). However, the prognosis of pCCA with curative resection is still dismal...
  3. Kriegsmann M, Roessler S, Kriegsmann K, Renner M, Longuespée R, Albrecht T, et al. Programmed cell death ligand 1 (PD-L1, CD274) in cholangiocarcinoma - correlation with clinicopathological data and comparison of antibodies. BMC Cancer. 2019;19:72 pubmed publisher
    ..Accordingly, highest PD-L1 positivity rates of stromal cells with 31% in iCCA, 40% in pCCA and 61% in dCCA were detected for clone SP263...
  4. Rojas J, Borden M, Dayton P. Effect of Hydrostatic Pressure, Boundary Constraints and Viscosity on the Vaporization Threshold of Low-Boiling-Point Phase-Change Contrast Agents. Ultrasound Med Biol. 2019;45:968-979 pubmed publisher
    ..In this study, the effect of boundary constraints, hydrostatic pressure and viscosity on PCCA vaporization pressure threshold are evaluated to explore possible mechanisms for variations in in vivo vaporization ..
  5. Perez B, Angaroni C, Sánchez Alcudia R, Merinero B, Perez Cerda C, Specola N, et al. The molecular landscape of propionic acidemia and methylmalonic aciduria in Latin America. J Inherit Metab Dis. 2010;33:S307-14 pubmed publisher
    ..In the PA patients, we have identified four different changes in the PCCA gene, including one novel one (c.414+5G>A) affecting the splicing process...
  6. Kaufmann R, Hascher A, Mussbach F, Henklein P, Katenkamp K, Westermann M, et al. Proteinase-activated receptor 2 (PAR(2)) in cholangiocarcinoma (CCA) cells: effects on signaling and cellular level. Histochem Cell Biol. 2012;138:913-24 pubmed publisher
    ..proteinase-activated receptor 2 (PAR(2)), a member of a G-protein receptor subfamily in primary cholangiocarcinoma (PCCA) cell cultures...
  7. Noakes R. Assessing the response of morphea and limited scleroderma to tranilast: a small prospective study comparing topical corticosteroids to a combination of topical corticosteroids and tranilast. Clin Cosmet Investig Dermatol. 2018;11:321-326 pubmed publisher
    ..All patients continued with their prescribed treatment and applied 0.1% betamethasone valerate in PCCA PracaSil™ (B) to the control site with 0...
  8. . Postconvulsive central apnea as a biomarker for sudden unexpected death in epilepsy (SUDEP). Neurology. 2019;92:e171-e182 pubmed publisher
    ..4%) seizures in 23 patients, all with focal epilepsy. Postconvulsive central apnea (PCCA) occurred in 31 of 140 (22.1%) seizures in 22 patients, with generalized, focal, or unknown epileptogenic zones...
  9. Rojas J, Dayton P. Vaporization Detection Imaging: A Technique for Imaging Low-Boiling-Point Phase-Change Contrast Agents with a High Depth of Penetration and Contrast-to-Tissue Ratio. Ultrasound Med Biol. 2019;45:192-207 pubmed publisher
    ..Furthermore, it was found that capturing PCCA vaporization signals produced higher contrast-to-tissue ratio values and better depth of penetration than imaging ..
  10. Ji G, Zhu F, Wang K, Xia Y, Jiao C, Shao Z, et al. Radiologic evaluation of vasculobiliary anatomy in the umbilical fissure. J Surg Res. 2017;214:254-261 pubmed publisher
    ..of vasculobiliary anatomy in the umbilical fissure (U-point) is pivotal for perihilar cholangiocarcinoma (PCCA) applied to right-sided hepatectomy...
  11. Hegazy M. A novel pure component contribution algorithm (PCCA) for extracting components' contribution from severely overlapped signals; an application to UV-spectrophotometric data. Spectrochim Acta A Mol Biomol Spectrosc. 2015;151:405-14 pubmed publisher
    ..The results were compared to previously reported separation method and no significant difference was found regarding both accuracy and precision. ..
  12. Chandler R, Chandrasekaran S, Carrillo Carrasco N, Senac J, Hofherr S, Barry M, et al. Adeno-associated virus serotype 8 gene transfer rescues a neonatal lethal murine model of propionic acidemia. Hum Gene Ther. 2011;22:477-81 pubmed publisher
    ..A murine model of PA (Pcca(-/-)) of animals that uniformly die within the first 48 hr of life was used to determine the efficacy of ..
  13. Déciga Campos M, Ortiz Andrade R, Sánchez Recillas A, Flores Guido J, Ramírez Camacho M. Antinociceptive and Antihyperalgesic Activity of a Traditional Maya Herbal Preparation Composed of Pouteria Campechiana, Chrysophyllum Cainito, Citrus Limonum, and Annona Muricata. Drug Dev Res. 2017;78:91-97 pubmed publisher
    ..cainito), Citrus limonum (C. limonum), and Annona muricata (A. muricata), that is commonly used in combination (PCCA) in traditional Mayan medicine for the treatment of diabetes and pain...
  14. Hady Cohen R, Ben Pazi H, Adir V, Yosovich K, Blumkin L, Lerman Sagie T, et al. Progressive cerebello-cerebral atrophy and progressive encephalopathy with edema, hypsarrhythmia and optic atrophy may be allelic syndromes. Eur J Paediatr Neurol. 2018;22:1133-1138 pubmed publisher
    ..2003, a new syndrome was described in the Sephardi Jewish population, named progressive cerebello-cerebral atrophy (PCCA) based on the typical neuroradiological findings...
  15. Zhu A. Future directions in the treatment of cholangiocarcinoma. Best Pract Res Clin Gastroenterol. 2015;29:355-61 pubmed publisher
    ..of biliary tract differentiation, and is best classified anatomically as intrahepatic CCA (ICC), perihilar (pCCA), or distal (dCCA) CCA. They represent a clinically and genetically diverse collection of cancers...
  16. Zayed H. Propionic acidemia in the Arab World. Gene. 2015;564:119-24 pubmed publisher
    ..manifestations, caused by a deficiency of propionyl-CoA carboxylase (PCC) enzyme, due to mutations in either PCCA or PCCB genes, which encode the alpha and beta subunits of the PCC enzyme, respectively...
  17. Akita M, Sofue K, Fujikura K, Otani K, Itoh T, Ajiki T, et al. Histological and molecular characterization of intrahepatic bile duct cancers suggests an expanded definition of perihilar cholangiocarcinoma. HPB (Oxford). 2019;21:226-234 pubmed publisher
    ..The present study aimed to elucidate how large-duct iCCA is similar and dissimilar to perihilar cholangiocarcinoma (pCCA). The study cohort consisted of iCCA (n = 58) and pCCA (n = 44)...
  18. Zawadzki K, Samecka Cymerman A, Kolon K, Wojtuń B, Mróz L, Kempers A. Metals in Pleurozium schreberi and Polytrichum commune from areas with various levels of pollution. Environ Sci Pollut Res Int. 2016;23:11100-8 pubmed publisher
    ..For P. commune, a steady decrease could be observed with increasing the distance up to 6000 m. PCCA ordination explained that both species from the vicinity of the chlor-alkali plant were correlated with the highest ..
  19. Youssef S, Hegazy M, Mohamed D, Badawey A. Analysis of paracetamol, pseudoephedrine and cetirizine in Allercet Cold® capsules using spectrophotometric techniques. Chem Cent J. 2018;12:67 pubmed publisher
    ..2, 257.0 and 242.4 nm, respectively. The fourth proposed method was pure component contribution algorithm (PCCA) which was applied to quantify the drugs at their ?max. Recovery percentages for RSDM were 100.7?±?1...
  20. Guenzel A, Collard R, Kraus J, Matern D, Barry M. Long-term sex-biased correction of circulating propionic acidemia disease markers by adeno-associated virus vectors. Hum Gene Ther. 2015;26:153-60 pubmed publisher
    Propionic academia (PA) occurs because of mutations in the PCCA or PCCB genes encoding the two subunits of propionyl-CoA carboxylase, a pivotal enzyme in the breakdown of certain amino acids and odd-chain fatty acids...
  21. Nakanuma Y, Kakuda Y. Pathologic classification of cholangiocarcinoma: New concepts. Best Pract Res Clin Gastroenterol. 2015;29:277-93 pubmed publisher
    ..Depending on anatomical location, CCA is classifiable as intrahepatic (iCCA), perihilar (pCCA), and distal CCA (dCCA)...
  22. Hou J, Xiang H, Han J. Propionyl coenzyme A (propionyl-CoA) carboxylase in Haloferax mediterranei: Indispensability for propionyl-CoA assimilation and impacts on global metabolism. Appl Environ Microbiol. 2015;81:794-804 pubmed publisher
    ..that the PCC enzyme consisted of a fusion protein of a biotin carboxylase and a biotin-carboxyl carrier protein (PccA [HFX_2490]), a carboxyltransferase component (PccB [HFX_2478]), and an essential small subunit (PccX [HFX_2479])...
  23. Chapman K, Collado M, Figler R, Hoang S, Armstrong A, Cui W, et al. Recapitulation of metabolic defects in a model of propionic acidemia using patient-derived primary hepatocytes. Mol Genet Metab. 2016;117:355-362 pubmed publisher
    ..is a disorder of intermediary metabolism with defects in the alpha or beta subunits of propionyl CoA carboxylase (PCCA and PCCB respectively) enzyme...
  24. Li M, Li K, Qi X, Wu W, Zheng L, He C, et al. Percutaneous Transhepatic Biliary Stent Implantation for Obstructive Jaundice of Perihilar Cholangiocarcinoma: A Prospective Study on Predictors of Stent Patency and Survival in 92 Patients. J Vasc Interv Radiol. 2016;27:1047-1055.e2 pubmed publisher
    To evaluate prognostic factors for stent patency and survival in patients with perihilar cholangiocarcinoma (pCCA) who underwent percutaneous biliary stent placement...
  25. Tebo A, Haven T, Jackson B. Autoantibody diversity in paraneoplastic syndromes and related disorders: The need for a more guided screening approach. Clin Chim Acta. 2016;459:162-169 pubmed publisher
    ..The positivity rates for non-classic PNS markers were 0% [AGNA and PCCA-Tr], and 0...
  26. Walter D, Herrmann E, Winkelmann R, Albert J, Liese J, Schnitzbauer A, et al. Role of CD15 expression in dysplastic and neoplastic tissue of the bile duct - a potential novel tool for differential diagnosis of indeterminate biliary stricture. Histopathology. 2016;69:962-970 pubmed publisher
    ..Tissue samples from patients with intrahepatic (iCCA, n = 22), perihilar (pCCA, n = 7) and distal CCA (dCCA, n = 15), who underwent surgical resection in the period from 2010 to 2015 were ..
  27. Jin C, Decker A, Harris D, Blough B. Effect of Substitution on the Aniline Moiety of the GPR88 Agonist 2-PCCA: Synthesis, Structure-Activity Relationships, and Molecular Modeling Studies. ACS Chem Neurosci. 2016;7:1418-1432 pubmed
    ..We previously reported that 2-PCCA was able to modulate GPR88-mediated cAMP production through a Gαi-coupled pathway...
  28. Eichstadt L, Corriveau L, Moore G, Knipp G, Cooper B, Gwin W. Absorption of Transdermal Fluoxetine Compounded in a Lipoderm Base Compared to Oral Fluoxetine in Client-owned Cats. Int J Pharm Compd. 2017;21:242-246 pubmed
    ..either oral (1 mg/kg) or transdermal (5 mg/kg; maximum 25 mg daily) fluoxetine compounded in a transdermal base (PCCA Lipoderm), administered daily for 60 days...
  29. Wojtuń B, Samecka Cymerman A, Kolon K, Kempers A. Metals in Racomitrium lanuginosum from Arctic (SW Spitsbergen, Svalbard archipelago) and alpine (Karkonosze, SW Poland) tundra. Environ Sci Pollut Res Int. 2018;25:12444-12450 pubmed publisher
    ..Principal component and classification analysis (PCCA) ordination revealed that mosses of Spitsbergen were distinguished by a significantly higher Na concentration of ..
  30. Loveday B, Knox J, Dawson L, Metser U, Brade A, Horgan A, et al. Neoadjuvant hyperfractionated chemoradiation and liver transplantation for unresectable perihilar cholangiocarcinoma in Canada. J Surg Oncol. 2018;117:213-219 pubmed publisher
    Neoadjuvant chemoradiation and liver transplantation may be offered for unresectable perihilar cholangiocarcinoma (pCCA). This study aimed to determine the dropout rate and survival of patients who entered a national tri-modality protocol...
  31. Cappuccio G, Atwal P, Donti T, Ugarte K, Merchant N, Craigen W, et al. Expansion of the Phenotypic Spectrum of Propionic Acidemia with Isolated Elevated Propionylcarnitine. JIMD Rep. 2017;35:33-37 pubmed publisher
    ..only mild elevations, all of whom were subsequently confirmed to have propionic acidemia by molecular analysis of PCCA and PCCB genes. To date, they have had a mild clinical course...
  32. Rizvi S, Gores G. Emerging molecular therapeutic targets for cholangiocarcinoma. J Hepatol. 2017;67:632-644 pubmed publisher
    ..CCAs are classified anatomically into intrahepatic (iCCA), perihilar (pCCA), and distal CCA (dCCA)...
  33. Kato T, Okumura I, Song S, Golby A, Hata N. Tendon-Driven Continuum Robot for Endoscopic Surgery: Preclinical Development and Validation of a Tension Propagation Model. IEEE ASME Trans Mechatron. 2015;20:2252-2263 pubmed
    ..The tip-to-tip error by FKM was significantly less than the one by conventional piecewise-constant-curvature approximation (PCCA) (FKM: 5.9 ± 2.9 mm vs. PCCA: 23.7 ± 3.6 mm, n=15, P < 0.01).
  34. Bere T, Dalu T, Mwedzi T. Detecting the impact of heavy metal contaminated sediment on benthic macroinvertebrate communities in tropical streams. Sci Total Environ. 2016;572:147-156 pubmed publisher
    ..Canonical correspondence analysis and partial canonical correspondence analysis (pCCA) were used to determine the importance of sediment heavy metal concentrations in explaining benthic ..
  35. Pramar Y, Graves R, Ledet G, Phan K, Bostanian L, Mandal T. Stability of Clindamycin Hydrochloride in PCCA Base SuspendIt. Int J Pharm Compd. 2016;20:421-425 pubmed
    ..The purpose of this study was to determine the chemical stability of clindamycin hydrochloride in the PCCA base SuspendIt, a sugar-free, paraben- free, dye-free, and gluten-free thixotropic vehicle containing a natural ..
  36. Fontugne J, Augustin J, Pujals A, Compagnon P, Rousseau B, Luciani A, et al. PD-L1 expression in perihilar and intrahepatic cholangiocarcinoma. Oncotarget. 2017;8:24644-24651 pubmed publisher
    ..Our goal was to characterize PD-L1 expression in intra-hepatic (iCCA) and perihilar (pCCA) cholangiocarcinomas, and to correlate our results with clinicopathological features, density of tumor-infiltrating ..
  37. Riemersma M, Hazebroek M, Helderman van den Enden A, Salomons G, Ferdinandusse S, Brouwers M, et al. Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017;25:1195-1201 pubmed publisher
    ..Genetic analysis demonstrated compound heterozygous variants in PCCA. Enzymatic activity of PCC in fibroblasts was markedly reduced...
  38. Chang Q, Martin L. Voltage-gated calcium channels are abnormal in cultured spinal motoneurons in the G93A-SOD1 transgenic mouse model of ALS. Neurobiol Dis. 2016;93:78-95 pubmed publisher
    ..Our study demonstrates that voltage-gated Ca(2+) channels have aberrant functions and localizations in ALS mouse motoneurons. The increased HVA Ca(2+) currents and PCCa current could contribute to early pathogenesis of ALS.
  39. Yan Z, Xue M, He Q, Lu W, Meng Z, Yan D, et al. A non-enzymatic urine glucose sensor with 2-D photonic crystal hydrogel. Anal Bioanal Chem. 2016;408:8317-8323 pubmed
    A novel polymerized crystalline colloidal array (PCCA) sensing material for the detection of urine glucose was developed by embedding a two-dimensional (2-D) polystyrene crystalline colloidal array (CCA) in 3-acrylamidophenylboronic acid (..
  40. Long H, Ge D, Zhang Y, Tu S, Qu Y, Ma L. Changing man-land interrelations in China's farming area under urbanization and its implications for food security. J Environ Manage. 2018;209:440-451 pubmed publisher
    ..two major coupling types between PCCT and PCFT, one is the double increasing of per capita construction land area (PCCA) and per capita farmland area (PCFA); another is the increasing of PCCA and the decreasing of PCFA...
  41. Wadhwani G. Homeopathic drug therapy. Homeopathy in Chikungunya Fever and Post-Chikungunya Chronic Arthritis: an observational study. Homeopathy. 2013;102:193-8 pubmed publisher
    To observe the effect of homeopathic therapy in Chikungunya Fever (CF) and in Post-Chikungunya Chronic Arthritis (PCCA) in a primary health care setting...
  42. Trasnea P, Utz M, Khalfaoui Hassani B, Lagies S, Daldal F, Koch H. Cooperation between two periplasmic copper chaperones is required for full activity of the cbb3 -type cytochrome c oxidase and copper homeostasis in Rhodobacter capsulatus. Mol Microbiol. 2016;100:345-61 pubmed publisher
    ..We identified the PCuA C-like periplasmic chaperone PccA and analyzed its contribution to cbb3 -Cox assembly...
  43. Marcano Bonilla L, Mohamed E, Mounajjed T, Roberts L. Biliary tract cancers: epidemiology, molecular pathogenesis and genetic risk associations. Chin Clin Oncol. 2016;5:61 pubmed publisher
    ..BTC are sub-classified as intrahepatic cholangiocarcinoma (iCCA), perhilar/hilar cholangiocarcinoma (pCCA), distal cholangiocarcinoma (dCCA), and gallbladder carcinoma...
  44. Decker A, Gay E, Mathews K, Rosa T, Langston T, Maitra R, et al. Development and validation of a high-throughput calcium mobilization assay for the orphan receptor GPR88. J Biomed Sci. 2017;24:23 pubmed publisher
    ..mobilization assay was validated by the structure-activity relationship study of known GPR88 agonist (1R,2R)-2-PCCA analogues...
  45. Unzueta A, Cabrera R. Treatment Options in Patients Awaiting Liver Transplantation with Hepatocellular Carcinoma and Cholangiocarcinoma. Clin Liver Dis. 2017;21:231-251 pubmed publisher
    ..a good chance of cure for selected patients with hepatocellular carcinoma (HCC) and perihilar cholangiocarcinoma (pCCA). Patients with HCC on a waiting list for LT are at risk for tumor progression and dropout...
  46. Jones N, Carvalho M, Branvold Herr A. Management of Psoriasis with a XemaTop Topical Compounded Formula: A Case Report. Int J Pharm Compd. 2017;21:205-211 pubmed
    ..clobetasol propionate, and cyanocobalamin in the Professional Compounding Centers of America's proprietary base PCCA XemaTop, applied topically for three weeks, significantly reduced the patient's self-reported psoriasis symptoms ..
  47. Nyankima A, Rojas J, Cianciolo R, Johnson K, Dayton P. In Vivo Assessment of the Potential for Renal Bio-Effects from the Vaporization of Perfluorocarbon Phase-Change Contrast Agents. Ultrasound Med Biol. 2018;44:368-376 pubmed publisher
    ..Results indicated that bio-effects from PCCA vaporization were not observed at lower mechanical indices...
  48. Chen P, Li B, Zhu Y, Chen W, Liu X, Li M, et al. Establishment and validation of a prognostic nomogram for patients with resectable perihilar cholangiocarcinoma. Oncotarget. 2016;7:37319-37330 pubmed publisher
    ..conventional staging systems have poor prognosis prediction ability for patients with perihilar cholangiocarcinoma (pCCA), we established and validated an effective prognostic nomogram for pCCA patients based on their personal and tumor ..
  49. Mowaka S, Hegazy M, Lotfy H, Mohamed E. Novel Pure Component Contribution Algorithm (PCCA) and UHPLC Methods for Separation and Quantification of Amlodipine, Valsartan, and Hydrochlorothiazide in Ternary Mixture. J AOAC Int. 2017;100:692-699 pubmed publisher
    ..The results were statistically compared to a previously reported method, and no significant difference was found regarding accuracy or precision. ..
  50. Mohammed D, Helal D. Prognostic significance of epithelial/stromal caveolin-1 expression in prostatic hyperplasia, high grade prostatic intraepithelial hyperplasia and prostatic carcinoma and its correlation with microvessel density. J Egypt Natl Canc Inst. 2017;29:25-31 pubmed publisher
    ..Stromal caveolin-1expression was significantly higher in BPH than HGPIN and PCca. There was significant inverse relation between stromal caveolin-1 expression and extension to lymph node and ..
  51. Dieho K, van Baal J, Kruijt L, Bannink A, Schonewille J, Carreno D, et al. Effect of supplemental concentrate during the dry period or early lactation on rumen epithelium gene and protein expression in dairy cattle during the transition period. J Dairy Sci. 2017;100:7227-7245 pubmed publisher
    ..In the lactation experiment, immunoblotting of 5 selected genes showed that protein expression of DRA and PCCA was greater at 16 d pp compared with 3 and 44 d pp...
  52. Lamhonwah A, Mahuran D, Gravel R. Human mitochondrial propionyl-CoA carboxylase: localization of the N-terminus of the pro- and mature alpha chains in the deduced primary sequence of a full-length cDNA. Nucleic Acids Res. 1989;17:4396 pubmed
  53. Lamhonwah A, Quan F, Gravel R. Sequence homology around the biotin-binding site of human propionyl-CoA carboxylase and pyruvate carboxylase. Arch Biochem Biophys. 1987;254:631-6 pubmed
    ..We propose that the 60 amino acids surrounding the biotin site are bounded by a proline "hinge" and the carboxy terminus has remained conserved as a result of constraints imposed by biotinylation of the enzyme. ..
  54. Gress T, Müller Pillasch F, Geng M, Zimmerhackl F, Zehetner G, Friess H, et al. A pancreatic cancer-specific expression profile. Oncogene. 1996;13:1819-30 pubmed
    ..In addition this has led to the identification of novel differentially expressed genes, which represent potential oncogenes or disease-associated markers and may be helpful for the development of therapeutic or diagnostic modalities. ..
  55. Muro S, Perez B, Desviat L, Rodríguez Pombo P, Perez Cerda C, Clavero S, et al. Effect of PCCB gene mutations on the heteromeric and homomeric assembly of propionyl-CoA carboxylase. Mol Genet Metab. 2001;74:476-83 pubmed
    ..of propionyl-CoA carboxylase, a dodecameric enzyme composed of alpha-PCC and beta-PCC subunits (encoded by genes PCCA and PCCB) that have been associated with a number of mutations responsible for this disease...
  56. Kozak M. Emerging links between initiation of translation and human diseases. Mamm Genome. 2002;13:401-10 pubmed
    ..Because the discovery of mutations that perturb translation usually begins with cDNA analysis, some misunderstandings vis-a-vis the interpretation of cDNA sequences are discussed. ..
  57. Clavero S, Martínez M, Perez B, Perez Cerda C, Ugarte M, Desviat L. Functional characterization of PCCA mutations causing propionic acidemia. Biochim Biophys Acta. 2002;1588:119-25 pubmed
    ..4.1.3), a heteropolymeric enzyme composed of alpha and beta subunits, which are encoded by the PCCA and PCCB genes, respectively...
  58. Yang X, Sakamoto O, Matsubara Y, Kure S, Suzuki Y, Aoki Y, et al. Mutation spectrum of the PCCA and PCCB genes in Japanese patients with propionic acidemia. Mol Genet Metab. 2004;81:335-42 pubmed
    ..This enzyme is composed of two non-identical subunits, alpha and beta, which are encoded by the PCCA and PCCB genes, respectively. An enzyme deficiency can result from mutations in either PCCA or PCCB...
  59. Rincon A, Aguado C, Desviat L, Sanchez Alcudia R, Ugarte M, Perez B. Propionic and methylmalonic acidemia: antisense therapeutics for intronic variations causing aberrantly spliced messenger RNA. Am J Hum Genet. 2007;81:1262-70 pubmed
    ..1957ins76), PCCA (r.1284ins84), or PCCB (r.654ins72) messenger RNAs (mRNAs)...
  60. Desviat L, Sánchez Alcudia R, Perez B, Perez Cerda C, Navarrete R, Vijzelaar R, et al. High frequency of large genomic deletions in the PCCA gene causing propionic acidemia. Mol Genet Metab. 2009;96:171-6 pubmed publisher
    Mutations in either the PCCA or PCCB genes are responsible for propionic acidemia (PA), one of the most frequent organic acidemias inherited in autosomal recessive fashion. Most of the mutations detected to date in both genes are missense...
  61. Vatanavicharn N, Liammongkolkul S, Sakamoto O, Kamolsilp M, Sathienkijkanchai A, Wasant P. Clinical characteristics and mutation analysis of propionic acidemia in Thailand. World J Pediatr. 2014;10:64-8 pubmed publisher
    ..The diagnosis of PA is confirmed by detection of mutations in the PCCA or PCCB genes. We herein report the clinical and molecular findings of four Thai patients with PA...
  62. Marshalek J, Sheeran P, Ingram P, Dayton P, Witte R, Matsunaga T. Intracellular delivery and ultrasonic activation of folate receptor-targeted phase-change contrast agents in breast cancer cells in vitro. J Control Release. 2016;243:69-77 pubmed publisher
    ..4% internalization at MI=1.0, 35.7% at MI=1.5). Folate incorporation increased the frequency of intracellular PCCA detection 45-fold for MDA-MB-231 cells and 7-fold for MCF-7 cells, relative to untargeted PCCAs...
  63. Rizvi S, Khan S, Hallemeier C, Kelley R, Gores G. Cholangiocarcinoma - evolving concepts and therapeutic strategies. Nat Rev Clin Oncol. 2017;: pubmed publisher
    ..cholangiocarcinomas are categorized according to anatomical location as intrahepatic (iCCA), perihilar (pCCA), or distal (dCCA)...
  64. Graves R, Phan K, Bostanian L, Mandal T, Pramar Y. Physicochemical Stability of an Oral Suspension of Trimethoprim 20 mg/mL in Combination with Sulfadiazine 200 mg/mL in PCCA Base SuspendIt. Int J Pharm Compd. 2017;21:430-435 pubmed
    ..purpose of this study was to determine the physicochemical stability of trimethoprim combined with sulfadiazine in PCCA base SuspendIt...
  65. Kennerknecht I, Klett C, Hameister H. Assignment of the human gene propionyl coenzyme A carboxylase, alpha-chain, (PCCA) to chromosome 13q32 by in situ hybridization. Genomics. 1992;14:550-1 pubmed
  66. Qi T, Wang W, Xu M. [A preliminary study on metastasis-associated gene screened by monoclonal antibodies HIL]. Zhonghua Zhong Liu Za Zhi. 1995;17:163-6 pubmed
    ..The metastatic potential of Anip[973] cells was markedly decreased after being pretreated with MoAb H4. The above findings indicate that H4-D has a certain relationship with the metastatic phenotype of Anip[973] cells. ..
  67. Lamhonwah A, Leclerc D, Loyer M, Clarizio R, Gravel R. Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit. Genomics. 1994;19:500-5 pubmed
    ..Mutations of the PCCA (alpha subunit) or PCCB (beta subunit) gene cause the inherited metabolic disease, propionic acidemia...
  68. Richard E, Desviat L, Perez B, Perez Cerda C, Ugarte M. Genetic heterogeneity in propionic acidemia patients with alpha-subunit defects. Identification of five novel mutations, one of them causing instability of the protein. Biochim Biophys Acta. 1999;1453:351-8 pubmed
    The inherited metabolic disease propionic acidemia (PA) can result from mutations in either of the genes PCCA or PCCB, which encode the alpha and beta subunits, respectively, of the mitochondrial enzyme propionyl CoA-carboxylase...
  69. Campeau E, Dupuis L, Leon del Rio A, Gravel R. Coding sequence mutations in the alpha subunit of propionyl-CoA carboxylase in patients with propionic acidemia. Mol Genet Metab. 1999;67:11-22 pubmed
    ..The results suggest that most mutations have a severe impact on the stability or the functionality of the alpha subunit. ..
  70. Campeau E, Desviat L, Leclerc D, Wu X, Perez B, Ugarte M, et al. Structure of the PCCA gene and distribution of mutations causing propionic acidemia. Mol Genet Metab. 2001;74:238-47 pubmed
    ..Affected patients may have mutations of either the PCCA or PCCB gene...
  71. Hassan Y, Moriyama H, Olsen L, Bi X, Zempleni J. N- and C-terminal domains in human holocarboxylase synthetase participate in substrate recognition. Mol Genet Metab. 2009;96:183-8 pubmed publisher
    ..In silico predictions were consistent with observations from Y2H assays and yeast rescue experiments, and suggested docking of p67 near Arg508 and Ser515 within the central domain of HCS. ..
  72. Huang C, Sadre Bazzaz K, Shen Y, Deng B, Zhou Z, Tong L. Crystal structure of the alpha(6)beta(6) holoenzyme of propionyl-coenzyme A carboxylase. Nature. 2010;466:1001-5 pubmed publisher
  73. Meierhofer D, Halbach M, Åžen N, Gispert S, Auburger G. Ataxin-2 (Atxn2)-Knock-Out Mice Show Branched Chain Amino Acids and Fatty Acids Pathway Alterations. Mol Cell Proteomics. 2016;15:1728-39 pubmed publisher
    ..In liver tissue, significant downregulations of the proteins ACADS, ALDH6A1, ALDH7A1, IVD, MCCC2, PCCA, OTC, together with bioinformatic enrichment of downregulated pathways for branched chain and other amino acid ..
  74. Jin C, Decker A, Langston T. Design, synthesis and pharmacological evaluation of 4-hydroxyphenylglycine and 4-hydroxyphenylglycinol derivatives as GPR88 agonists. Bioorg Med Chem. 2017;25:805-812 pubmed publisher
    ..GPR88 couples to G?i proteins and modulates cAMP levels upon treatment with a small molecule agonist 2-PCCA. Recently, another chemotype of GPR88 agonist, represented by 2-AMPP [(2S)-N-((1R)-2-amino-1-(4-(2-methylpentyloxy)-..
  75. Chapman K, Ostrovsky J, Rao M, Dingley S, Polyak E, Yudkoff M, et al. Propionyl-CoA carboxylase pcca-1 and pccb-1 gene deletions in Caenorhabditis elegans globally impair mitochondrial energy metabolism. J Inherit Metab Dis. 2018;41:157-168 pubmed publisher
    ..PA is inherited in an autosomal recessive fashion due to functional loss of both alleles of either PCCA or PCCB...
  76. Lamhonwah A, Barankiewicz T, Willard H, Mahuran D, Quan F, Gravel R. Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes. Proc Natl Acad Sci U S A. 1986;83:4864-8 pubmed
    ..By use of a panel of somatic mouse-human hybrids, the human gene encoding the alpha polypeptide (PCCA) was localized to chromosome 13, while the gene encoding the beta polypeptide (PCCB) was assigned to chromosome 3...
  77. Lamhonwah A, Gravel R. Propionicacidemia: absence of alpha-chain mRNA in fibroblasts from patients of the pccA complementation group. Am J Hum Genet. 1987;41:1124-31 pubmed
    ..From these data, we confirm the gene assignments of the complementation groups (PCCA gene = pccA complementation group; PCCB gene = pccBC and subgroups) and support the view that pccA patients synthesize a normal beta chain ..
  78. Perez B, Desviat L, Rodríguez Pombo P, Clavero S, Navarrete R, Perez Cerda C, et al. Propionic acidemia: identification of twenty-four novel mutations in Europe and North America. Mol Genet Metab. 2003;78:59-67 pubmed
    ..PCC is a multimeric protein composed of two different alpha- and beta-PCC subunits, nuclearly encoded by the PCCA and PCCB genes, respectively...
  79. Huen M, Huang J, Yuan J, Yamamoto M, Akira S, Ashley C, et al. Noncanonical E2 variant-independent function of UBC13 in promoting checkpoint protein assembly. Mol Cell Biol. 2008;28:6104-12 pubmed publisher
    ..We propose that the RNF8 RING domain selects and loads a subset of UBC13 molecules, distinct from those that exist as heterodimers, onto sites of double-strand breaks, which facilitates the amplification of DNA damage signals. ..
  80. MacDonald M, Longacre M, Langberg E, Tibell A, Kendrick M, Fukao T, et al. Decreased levels of metabolic enzymes in pancreatic islets of patients with type 2 diabetes. Diabetologia. 2009;52:1087-91 pubmed publisher
    ..Low levels of certain islet metabolic enzymes, especially mitochondrial enzymes, are associated with human type 2 diabetes. ..
  81. Chen Z, Wen P, Wang G, Hu Y, Liu X, Chen L, et al. [Analysis of PCCA and PCCB gene mutations in patients with propionic acidemia]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2015;32:26-30 pubmed publisher
    To analyze PCCA and PCCB gene mutations in 10 Chinese patients with propionic acidemia(PA). Genomic DNA was extracted from peripheral blood leukocytes...
  82. Graves R, Phan K, Bostanian L, Mandal T, Pramar Y. Stability of Spironolactone Oral Suspension in PCCA Base, SuspendIt. Int J Pharm Compd. 2017;21:334-338 pubmed
    ..The purpose of this study was to determine the physicochemical stability of spironolactone in the PCCA base SuspendIt...
  83. Mertens J, Rizvi S, Gores G. Targeting cholangiocarcinoma. Biochim Biophys Acta Mol Basis Dis. 2018;1864:1454-1460 pubmed publisher
    ..associated with the biliary tract, and can best be stratified anatomically into intrahepatic (iCCA), perihilar (pCCA) and distal (dCCA) subsets...
  84. Alonso Barroso E, Brasil S, Briso Montiano Á, Navarrete R, Perez Cerda C, Ugarte M, et al. Generation and characterization of a human iPSC line from a patient with propionic acidemia due to defects in the PCCA gene. Stem Cell Res. 2017;23:173-177 pubmed publisher
    ..stem cell (iPSC) line was generated from fibroblasts of a patient with propionic acidemia carrying mutations in the PCCA gene: c.1899+4_1899+7delAGTA; p.(Cys616_Val633del) and c.1430--?_1643+?del; p.(Gly477Glufs*9)...
  85. Stankovics J, Ledley F. Cloning of functional alpha propionyl CoA carboxylase and correction of enzyme deficiency in pccA fibroblasts. Am J Hum Genet. 1993;52:144-51 pubmed
    Propionyl CoA carboxylase (PPC) is a heteromeric enzyme composed of alpha subunits (PCCA) and beta (PCCB) subunits...
  86. Ugarte M, Perez Cerda C, Rodríguez Pombo P, Desviat L, Perez B, Richard E, et al. Overview of mutations in the PCCA and PCCB genes causing propionic acidemia. Hum Mutat. 1999;14:275-82 pubmed
    ..The enzyme is composed of alpha and beta subunits which are encoded by the PCCA and PCCB genes, respectively. Mutations in both genes can cause propionic acidemia...
  87. Chiu Y, Liu Y, Liao W, Chang Y, Lin S, Hsu C, et al. Two frequent mutations associated with the classic form of propionic acidemia in Taiwan. Biochem Genet. 2014;52:415-29 pubmed publisher
    ..PCC consists of two subunits, ? and ?, encoded by the PCCA and PCCB genes, respectively. Mutations in the PCCA or PCCB subunit gene may lead to propionic acidemia...
  88. Gupta D, Bijarnia Mahay S, Kohli S, Saxena R, Puri R, Shigematsu Y, et al. Seventeen Novel Mutations in PCCA and PCCB Genes in Indian Propionic Acidemia Patients, and Their Outcomes. Genet Test Mol Biomarkers. 2016;20:373-82 pubmed publisher
    The goal of this study was to identify mutations in the propionyl-CoA carboxylase alpha subunit (PCCA) and propionyl-CoA carboxylase beta subunit (PCCB) genes, and to assess their effects on propionic academia (PA) patients...
  89. Wongkittichote P, Ah Mew N, Chapman K. Propionyl-CoA carboxylase - A review. Mol Genet Metab. 2017;122:145-152 pubmed publisher
    ..is the enzyme which catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form a hetero-dodecamer...
  90. Chloupkova M, Ravn K, Schwartz M, Kraus J. Changes in the carboxyl terminus of the beta subunit of human propionyl-CoA carboxylase affect the oligomer assembly and catalysis: expression and characterization of seven patient-derived mutant forms of PCC in Escherichia coli. Mol Genet Metab. 2000;71:623-32 pubmed
    ..Human PCC is a dodecamer composed of pairs of nonidentical alpha and beta subunits encoded by PCCA and PCCB genes, respectively...