Gene Symbol: BCKDHB
Description: branched chain keto acid dehydrogenase E1 subunit beta
Alias: BCKDE1B, BCKDH E1-beta, E1B, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, E1b-beta subunit of the branched-chain complex, branched chain alpha-ketoacid dehydrogenase E1-beta subunit, branched chain keto acid dehydrogenase E1, beta polypeptide, branched-chain alpha-keto acid dehydrogenase E1 component beta chain, testis secretory sperm-binding protein Li 240mP
Species: human
Products:     BCKDHB

Top Publications

  1. Abiri M, Talebi S, Uitto J, Youssefian L, Vahidnezhad H, Shirzad T, et al. Co-existence of phenylketonuria either with maple syrup urine disease or Sandhoff disease in two patients from Iran: emphasizing the role of consanguinity. J Pediatr Endocrinol Metab. 2016;29:1215-1219 pubmed publisher
    ..Ser231Pro and p.Ala300Ser in the PAH gene and p.Glu330Lys and p.Arg170Cys mutations in the BCKDHB gene. Genetic testing results in the second patient showed previously reported homozygous mutations of p...
  2. Nakagaki A, Urakawa A, Hirano S, Anami T, Kishino T. Application of droplet digital PCR in the analysis of genome integration and organization of the transgene in BAC transgenic mice. Sci Rep. 2018;8:6638 pubmed publisher
    ..of five fragments of the BAC clone containing the Mkrn3 gene region, and that the transgene was inserted into Bckdhb, homozygous deletion of which causes the maple syrup urine disease phenotype...
  3. Soler Torronteras R, Lara Chica M, Garcia V, Calzado M, Munoz E. Hypoximimetic activity of N-acyl-dopamines. N-arachidonoyl-dopamine stabilizes HIF-1? protein through a SIAH2-dependent pathway. Biochim Biophys Acta. 2014;1843:2730-43 pubmed publisher
    ..EPO), vascular endothelial growth factor A (VEGFA), heme oxygenase 1 (HMOX-1), hexokinase 2 (HK2) and Bcl-2/E1B-nineteen kiloDalton interacting protein (BNIP3) in primary astrocytes...
  4. Chavez Valdez R, Martin L, Flock D, Northington F. Necrostatin-1 attenuates mitochondrial dysfunction in neurons and astrocytes following neonatal hypoxia-ischemia. Neuroscience. 2012;219:192-203 pubmed publisher
    ..Nec-1 following HI prevented: (i) up-regulation of hypoxia inducible factor-1 alpha (HIF-1?) and BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3) expression, (ii) decline in mitochondrial complex-I activity, (iii) ..
  5. Newell C, Shutt T, Ahn Y, Hittel D, Khan A, Rho J, et al. Tissue Specific Impacts of a Ketogenic Diet on Mitochondrial Dynamics in the BTBRT+tf/j Mouse. Front Physiol. 2016;7:654 pubmed publisher
    ..To this end, we examined expression levels of the mitophagy regulator BNIP3 (BCL2/adenovirus E1B 19 kd-interacting protein 3)...
  6. Tsou Y, Wang H, Hsieh C, Sun K, Sun G, Jhou R, et al. Down-regulation of BNIP3 by olomoucine, a CDK inhibitor, reduces LPS- and NO-induced cell death in BV2 microglial cells. Neurosci Lett. 2016;628:186-93 pubmed publisher
    ..Microarray analysis revealed that olomoucine treatment induced prominent down-regulation of Bcl2/adenovirus E1B 19-kDa-interacting protein 3 (BNIP3), a pro-apoptotic Bcl-2 family protein that is involved in mitochondrial ..
  7. Wu H, Chen Q. Hypoxia activation of mitophagy and its role in disease pathogenesis. Antioxid Redox Signal. 2015;22:1032-46 pubmed publisher
    ..Mitophagy receptors include Atg32 in yeast, as well as NIX/BNIP3L, B-cell lymphoma 2/adenovirus E1B 19-kDa-interacting protein 3 and FUN14 domain containing 1 in mammals...
  8. Guénard F, Bouchard Mercier A, Rudkowska I, Lemieux S, Couture P, Vohl M. Genome-Wide Association Study of Dietary Pattern Scores. Nutrients. 2017;9: pubmed publisher
    ..assessment of SNPs demonstrated an impact on gene expression levels in blood for SNPs located within/near BCKDHB (p = 0.02) and the hypothalamic glucosensor PFKFB3 (p = 0...
  9. Ameri K, Luong R, Zhang H, Powell A, Montgomery K, Espinosa I, et al. Circulating tumour cells demonstrate an altered response to hypoxia and an aggressive phenotype. Br J Cancer. 2010;102:561-9 pubmed publisher
    ..Hypoxia induced lower levels of carbonic anhydrase IX (CAIX), GLUT1 and BCL2/adenovirus E1B 19-KD protein-interacting protein 3 (BNIP3) proteins in CTCs than in parental cells, supporting an altered hypoxia ..

More Information

Publications136 found, 100 shown here

  1. Zhang J, Wu K, Xiao X, Liao J, Hu Q, Chen H, et al. Autophagy as a regulatory component of erythropoiesis. Int J Mol Sci. 2015;16:4083-94 pubmed publisher
    ..summarized, including autophagy-related (Atg) genes, the B-cell lymphoma 2 (Bcl-2) family member Bcl-2/adenovirus E1B 19 kDa interacting protein 3-like (Nix/Binp3L), transcription factors globin transcription factor 1 (GATA1) and ..
  2. Matsubara S, Bokuda K, Asano Y, Morishima R, Sugaya K, Miyamoto K, et al. Mitophagy in three cases of immune-mediated necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies: ultrastructural and immunohistochemical studies. Neuromuscul Disord. 2018;28:283-288 pubmed publisher
    ..Furthermore, upregulation of B-cell lymphoma 2/adenovirus E1B 19 kD-interacting protein 3 (BNIP3), a protein involved in mitophagy, was observed in two cases examined...
  3. O Sullivan T, Johnson L, Kang H, Sun J. BNIP3- and BNIP3L-Mediated Mitophagy Promotes the Generation of Natural Killer Cell Memory. Immunity. 2015;43:331-42 pubmed publisher
    ..Furthermore, we demonstrated a temporally regulated role for mitophagy-inducing proteins BCL2/adenovirus E1B 19-kDa interacting protein 3 (BNIP3) and BNIP3-like (BNIP3L) in the generation of robust NK cell memory...
  4. Wu H, Huang S, Chen Z, Liu W, Zhou X, Zhang D. Hypoxia-induced autophagy contributes to the invasion of salivary adenoid cystic carcinoma through the HIF-1α/BNIP3 signaling pathway. Mol Med Rep. 2015;12:6467-74 pubmed publisher
    ..The hypoxia‑inducible factor 1α/B cell lymphoma 2/adenovirus E1B 19K‑interacting protein 3 signaling pathway was involved in hypoxia‑induced autophagy in ACC...
  5. Atwan Z, Wright J, Woodman A, Leppard K. Promyelocytic leukemia protein isoform II inhibits infection by human adenovirus type 5 through effects on HSP70 and the interferon response. J Gen Virol. 2016;97:1955-67 pubmed publisher
    ..to effects of HSP70 on major late promoter or L4 promoter activity, but might be linked to an observed increase in E1B 55K, as this protein is known to be required for efficient late gene expression...
  6. Chang W, Huang H, Huang W, Chen C, Chen Y. Armillaridin induces autophagy-associated cell death in human chronic myelogenous leukemia K562 cells. Tumour Biol. 2016;37:14291-14300 pubmed
    ..autophagosome induction, the processing of autophagosome-lysosome fusion and downregulation of BCL2/adenovirus E1B 19 kDa interacting protein 3 (BNIP3)...
  7. Luan Y, Zhang X, Zhang Y, Dong Y. MicroRNA-210 Protects PC-12 Cells Against Hypoxia-Induced Injury by Targeting BNIP3. Front Cell Neurosci. 2017;11:285 pubmed publisher
    ..Overexpression of miR-210 protected PC-12 cells against hypoxia-induced injury. Bcl-2 adenovirus E1B 19 kDa-interacting protein 3 (BNIP3) was proven to be a target gene of miR-210 in PC-12 cells...
  8. Lu J, Jiang Z, Chen Y, Zhou C, Chen C. Knockout of programmed cell death 5 (PDCD5) gene attenuates neuron injury after middle cerebral artery occlusion in mice. Brain Res. 2016;1650:152-161 pubmed publisher
    ..expression of VHL may result in increased expression of hypoxia-inducible factor 1?(HIF-1?) and (BCL2/adenovirus E1B 19kDa protein-interacting protein 3) BNIP3...
  9. Li H, Wang X, Rukina D, Huang Q, Lin T, Sorrentino V, et al. An Integrated Systems Genetics and Omics Toolkit to Probe Gene Function. Cell Syst. 2017;: pubmed publisher
    ..analysis we established regulatory relations between genes, such as the co-regulation of BCKDHA and BCKDHB protein levels, and identified targets of transcription factors E2F6, ZFP277, and ZKSCAN1...
  10. Jiang K, Wang W, Jin X, Wang Z, Ji Z, Meng G. Silibinin, a natural flavonoid, induces autophagy via ROS-dependent mitochondrial dysfunction and loss of ATP involving BNIP3 in human MCF7 breast cancer cells. Oncol Rep. 2015;33:2711-8 pubmed publisher
    ..Silibinin stimulated the expression of Bcl-2 adenovirus E1B 19-kDa-interacting protein 3 (BNIP3), a pro-death Bcl-2 family member, and silencing of BNIP3 greatly inhibited ..
  11. Guo Y, Deng X, Chen S, Yang L, Ni J, Wang R, et al. MicroRNA-30e targets BNIP3L to protect against aldosterone-induced podocyte apoptosis and mitochondrial dysfunction. Am J Physiol Renal Physiol. 2017;312:F589-F598 pubmed publisher
    ..Next, we found that miR-30e could directly target the BCL2/adenovirus E1B-interacting protein 3-like (BNIP3L) gene...
  12. Natarajan K, Xie Y, Nakanishi T, Moreci R, Jeyasuria P, Hussain A, et al. Methods to Discover Alternative Promoter Usage and Transcriptional Regulation of Murine Bcrp1. J Vis Exp. 2016;: pubmed publisher
    ..alternative promoters that are designated by the corresponding four alternative first exons produced: E1U, E1A, E1B, and E1C. Herein, in-silico protocols are presented to predict alternative promoter usage for Bcrp1...
  13. Yoo N, Kim M, Park S, Seo S, Song S, Lee J, et al. Expression analysis of caspase-6, caspase-9 and BNIP3 in prostate cancer. Tumori. 2010;96:138-42 pubmed
    ..The aim of this study was to explore whether the expression of the proapoptotic proteins caspase-6, caspase-9, and Bcl-2/adenovirus E1B-19kDa-interacting protein3 (BNIP3) is altered in prostate cancers.
  14. Lee H, Jung Y, Choi G, Ko S, Lee S, Lee S, et al. BNIP3 induction by hypoxia stimulates FASN-dependent free fatty acid production enhancing therapeutic potential of umbilical cord blood-derived human mesenchymal stem cells. Redox Biol. 2017;13:426-443 pubmed publisher
    ..This study revealed that Bcl2/adenovirus E1B 19kDa protein-interacting protein 3 (BNIP3)-dependent mitophagy is important for reducing mitochondrial reactive ..
  15. Trollmann R, Richter M, Jung S, Walkinshaw G, Brackmann F. Pharmacologic stabilization of hypoxia-inducible transcription factors protects developing mouse brain from hypoxia-induced apoptotic cell death. Neuroscience. 2014;278:327-42 pubmed publisher
    ..ADM), erythropoietin (EPO), inducible nitric oxide synthase (iNOS)) as well as pro-apoptotic (BCL2/adenovirus E1B 19-kDa protein-interacting protein 3 gene (BNIP3), immediate early response 3 (IER3)) and migratory factors (..
  16. Janmohamed S, Brinkhuizen T, Den Hollander J, Madern G, de Laat P, van Steensel M, et al. Support for the hypoxia theory in the pathogenesis of infantile haemangioma. Clin Exp Dermatol. 2015;40:431-7 pubmed publisher
    ..in paraffin wax) were immunohistochemically analysed for HIF1α and the known HIF1α targets: BCL2/adenovirus E1B kD-interacting protein family member 3 (BNIP3), carbon anhydrase (CA)-IX, glucose transporter (GLUT)-1, ..
  17. Subramanian T, Vijayalingam S, Kuppuswamy M, Chinnadurai G. Interaction of cellular proteins with BCL-xL targeted to cytoplasmic inclusion bodies in adenovirus infected cells. Virology. 2015;483:21-31 pubmed publisher
    ..apoptosis was suppressed when cellular anti-apoptosis proteins (BCL-2 and BCL-xL) were substituted for the viral E1B-19K...
  18. Shalitanati A, Yu H, Liu D, Xu W, Yue X, Guo R, et al. Fine mapping epitope on glycoprotein-Gn from Crimean-Congo hemorrhagic fever virus. Comp Immunol Microbiol Infect Dis. 2018;59:24-31 pubmed publisher
    ..The eight fine BCEs (Gn-E1a, 543RTQLV547; E1b, 553EIH555; E1c, 554IHEDSY559; E1d, 557DSYG560; ..
  19. Song D, Li W, Wei C, Wang W, Lyu J. [A classic case with maple syrup urine disease caused by compound heterozygous mutations of BCKDHB gene]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2018;35:699-702 pubmed publisher
    ..Next-generation sequencing (NGS) was used to screen the exons of BCKDHA, BCKDHB, DBT and DLD genes. Suspected mutations were verified by Sanger sequencing...
  20. Shen Y, Gong X, Yan J, Qin L, Qiu G. [Maple syrup urine disease caused by two novel BCKDHB gene mutations in a Chinese neonate]. Zhonghua Er Ke Za Zhi. 2015;53:66-70 pubmed
    ..ethnic Chinese newborn infant with the severe classic form of MSUD caused by two novel missense mutations in the BCKDHB gene...
  21. Esteban Martínez L, Sierra Filardi E, McGreal R, Salazar Roa M, Mariño G, Seco E, et al. Programmed mitophagy is essential for the glycolytic switch during cell differentiation. EMBO J. 2017;36:1688-1706 pubmed publisher
    ..Local hypoxia triggered expression of the mitophagy regulator BCL2/adenovirus E1B 19-kDa-interacting protein 3-like (BNIP3L, best known as NIX) at peak RGC differentiation...
  22. Reichel A, Stilp A, Scherer M, Reuter N, Lukassen S, Kasmapour B, et al. Chromatin-Remodeling Factor SPOC1 Acts as a Cellular Restriction Factor against Human Cytomegalovirus by Repressing the Major Immediate Early Promoter. J Virol. 2018;92: pubmed publisher
    ..studies identified SPOC1 as a restriction factor against human adenovirus (HAdV) infection that is antagonized by E1B-55K/E4-orf6-dependent proteasomal degradation...
  23. Turner M, Middha S, Hofherr S, Barry M. Comparison of the Life Cycles of Genetically Distant Species C and Species D Human Adenoviruses Ad6 and Ad26 in Human Cells. J Virol. 2015;89:12401-17 pubmed publisher
    ..There were marked differences in the balance of E1A and E1B activation by the two viruses and in the expression of E3 immune evasion mRNAs...
  24. Brahimi Horn M, Lacas Gervais S, Adaixo R, Ilc K, Rouleau M, Notte A, et al. Local mitochondrial-endolysosomal microfusion cleaves voltage-dependent anion channel 1 to promote survival in hypoxia. Mol Cell Biol. 2015;35:1491-505 pubmed publisher
    ..demonstrated that hypoxia-inducible factor 1 mediates the hyperfusion of mitochondria by inducing Bcl-2/adenovirus E1B 19-kDa interacting protein 3 and posttranslational truncation of the mitochondrial ATP transporter outer membrane ..
  25. Yan X, Sun Y, Ren S, Zhao L, Liu W, Chen C, et al. Dietary ?-Mangostin Provides Protective Effects against Acetaminophen-Induced Hepatotoxicity in Mice via Akt/mTOR-Mediated Inhibition of Autophagy and Apoptosis. Int J Mol Sci. 2018;19: pubmed publisher
    ..the protein expression of autophagy-related microtubule-associated protein light chain 3 (LC3) and BCL2/adenovirus E1B protein-interacting protein 3 (BNIP3)...
  26. Liu X, Zhang W, Wu Z, Yang Y, Kang Y. Copper levels affect targeting of hypoxia-inducible factor 1α to the promoters of hypoxia-regulated genes. J Biol Chem. 2018;293:14669-14677 pubmed publisher
    ..It controls the expression of both BCL2/adenovirus E1B 19-kDa protein-interacting protein 3 (BNIP3) and insulin-like growth factor 2 (IGF2)...
  27. Ingemarsdotter C, Tookman L, Browne A, Pirlo K, Cutts R, Chelela C, et al. Paclitaxel resistance increases oncolytic adenovirus efficacy via upregulated CAR expression and dysfunctional cell cycle control. Mol Oncol. 2015;9:791-805 pubmed publisher
    ..The activity of multiple Ad5 vectors, including dl922-947 (E1A CR2-deleted), dl1520 (E1B-55K deleted) and Ad5 WT, was significantly increased in paclitaxel resistant ovarian cancer in vitro and in vivo...
  28. Liu J, Yuan C, Pu L, Wang J. Nutrient deprivation induces apoptosis of nucleus pulposus cells via activation of the BNIP3/AIF signalling pathway. Mol Med Rep. 2017;16:7253-7260 pubmed publisher
    ..model under ND conditions to investigate the roles of the nutrient?sensitive protein B?cell lymphoma 2/adenovirus E1B 19 kDa?interacting protein (BNIP3) and the mitochondrial pro?death protein apoptosis?inducing factor (AIF) in the ..
  29. Wimmer P, Berscheminski J, Blanchette P, Groitl P, Branton P, Hay R, et al. PML isoforms IV and V contribute to adenovirus-mediated oncogenic transformation by functionally inhibiting the tumor-suppressor p53. Oncogene. 2016;35:69-82 pubmed publisher
    Although modulation of the cellular tumor-suppressor p53 is considered to have the major role in E1A/E1B-55K-mediated tumorigenesis, other promyelocytic leukemia nuclear body (PML-NB)/PML oncogenic domain (POD)-associated factors ..
  30. Ma G, Liu Y, Zhang Q, Zhang B, Zhao N, Wang Q, et al. Pre-endurance training prevents acute alcoholic liver injury in rats through the regulation of damaged mitochondria accumulation and mitophagy balance. Hepatol Int. 2014;8:425-35 pubmed publisher
    ..The rats were examined to determine the following: BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3), hypoxia-inducible factor-1α (HIF-1α), cytochrome P450 2E1 (..
  31. Lee Y, Kubli D, Hanna R, Cortez M, Lee H, Miyamoto S, et al. Cellular redox status determines sensitivity to BNIP3-mediated cell death in cardiac myocytes. Am J Physiol Cell Physiol. 2015;308:C983-92 pubmed publisher
    The atypical BH3-only protein Bcl-2/adenovirus E1B 19-kDa interacting protein 3 (BNIP3) is an important regulator of hypoxia-mediated cell death. Interestingly, the susceptibility to BNIP3-mediated cell death differs between cells...
  32. Wang J, Gao Q, Li Q. Adenovirus type 12 E1B 55-kilodalton oncoprotein promotes p53-mediated apoptotic response of ovarian cancer to cisplatin. Tumour Biol. 2015;36:6569-77 pubmed publisher
    ..The adenovirus (Ad) type 12 E1B 55-kDa protein binds to p53 and inactivates its transcriptional transactivation function...
  33. Abiri M, Karamzadeh R, Karimipoor M, Ghadami S, Alaei M, Bagheri S, et al. Identification of six novel mutations in Iranian patients with maple syrup urine disease and their in silico analysis. Mutat Res. 2016;786:34-40 pubmed publisher
    ..Different types of disease causing mutations have been previously reported in BCKDHA, BCKDHB, DBT and DLD genes known to be responsible for MSUD phenotype...
  34. Abiri M, Karamzadeh R, Mojbafan M, Alaei M, Jodaki A, Safi M, et al. In silico analysis of novel mutations in maple syrup urine disease patients from Iran. Metab Brain Dis. 2017;32:105-113 pubmed publisher
    ..The disease is mainly caused by mutations either in the BCKDHA, BCKDHB, DBT or DLD genes encoding components of the E1α, E1β, E2 and E3 subunits of branched-chain α-keto acid ..
  35. Han B, Han B, Guo B, Liu Y, Cao Z. Two novel mutations in the BCKDHB gene that cause maple syrup urine disease. Pediatr neonatol. 2018;59:515-519 pubmed publisher
    ..Mutations in the three genes (BCKDHA, BCKDHB and DBT) are associated with MSUD...
  36. Jenner A, Yun C, Yoon A, Kim P, Coster A. Modelling heterogeneity in viral-tumour dynamics: The effects of gene-attenuation on viral characteristics. J Theor Biol. 2018;454:41-52 pubmed publisher
    ..how modifications to the viral genome alter the viral characteristics, specifically how the attenuation of the E1B 19 and E1B 55 gene affect the system performance, and identify the dominant processes altered by the mutations...
  37. Liu X, Huang Z, Zou X, Yang Y, Qiu Y, Wen Y. Possible mechanism of PNS protection against cisplatin-induced nephrotoxicity in rat models. Toxicol Mech Methods. 2015;25:347-54 pubmed publisher
    ..PNS) against cisplatin-induced nephrotoxicity via the hypoxia inducible factor 1 (HIF-1)/Bcl-2/adenovirus E1B 19 kDa-interacting protein 3 (BNIP3) pathway of autophagy...
  38. Chen Z, Wu H, Huang S, Li W, Zhang S, Zheng P, et al. Expression of BNIP3 and its correlations to hypoxia-induced autophagy and clinicopathological features in salivary adenoid cystic carcinoma. Cancer Biomark. 2015;15:467-75 pubmed publisher
    The expression of Bcl-2/adenovirus E1B 19 kDa-interacting protein3 (BNIP3) has been explored in many human malignancies, but not in adenoid cystic carcinoma (ACC)...
  39. Gilbert R, Guilbault C, Gagnon D, Bernier A, Bourget L, Elahi S, et al. Establishment and validation of new complementing cells for production of E1-deleted adenovirus vectors in serum-free suspension culture. J Virol Methods. 2014;208:177-88 pubmed publisher
    ..Amplification of AdV must take place in cells that express the adenovirus E1A and E1B genes...
  40. Van Damme P, Plasman K, Vandemoortele G, Jonckheere V, Maurer Stroh S, Gevaert K. Importance of extended protease substrate recognition motifs in steering BNIP-2 cleavage by human and mouse granzymes B. BMC Biochem. 2014;15:21 pubmed publisher
    ..an identical P4-P1 human Bid (hBid) cleavage motif, mGrB was shown to efficiently cleave the BCL2/adenovirus E1B 19 kDa protein-interacting protein 2 or BNIP-2 at IEAD28...
  41. Higginbotham J, O Shea C. Adenovirus E4-ORF3 Targets PIAS3 and Together with E1B-55K Remodels SUMO Interactions in the Nucleus and at Virus Genome Replication Domains. J Virol. 2015;89:10260-72 pubmed publisher
    Adenovirus E4-ORF3 and E1B-55K converge in subverting critical overlapping cellular pathways to facilitate virus replication...
  42. Liu H, Zhu H, Li T, Zhang P, Wang N, Sun X. Prolyl-4-Hydroxylases Inhibitor Stabilizes HIF-1? and Increases Mitophagy to Reduce Cell Death After Experimental Retinal Detachment. Invest Ophthalmol Vis Sci. 2016;57:1807-15 pubmed publisher
    ..The mitophagy-related markers Hypoxia Inducible Factor 1? (HIF-1?), BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3), autophagy-related gene 5 (Atg5), microtubule-associated protein 1 ..
  43. Ham D, Kennedy T, Caldow M, Chee A, Lynch G, Koopman R. Citrulline does not prevent skeletal muscle wasting or weakness in limb-casted mice. J Nutr. 2015;145:900-6 pubmed publisher
    ..the nitric oxide synthase 2, inducible (Nos2; ~15-fold) and B-cell chronic lymphoid leukemia/lymphoma 2/adenovirus E1B 19 kDa interacting protein 3 (Bnip3; ~17-fold) genes and the ratio of microtubule-associated protein light chain ..
  44. He Z, Pu L, Yuan C, Jia M, Wang J. Nutrition deficiency promotes apoptosis of cartilage endplate stem cells in a caspase-independent manner partially through upregulating BNIP3. Acta Biochim Biophys Sin (Shanghai). 2017;49:25-32 pubmed publisher
    ..Bcl-2/adenovirus E1B 19-kDa-interacting protein 3 (BNIP3) has been reported to regulate apoptosis, autophagy, and cytoprotection...
  45. Li Q, Wang J, Liao D, Ai J, Jin L, Gao Q. Degradation of DAXX by adenovirus type 12 E1B-55K circumvents chemoresistance of ovarian cancer to cisplatin. Virology. 2018;521:118-128 pubmed publisher
    Adenovirus E1B 55-kilodalton (E1B-55K) mediated DAXX degradation represents a potential mechanism by which E1B-55K sensitizes cancer cells to chemotherapy...
  46. HUNG G, Flint S. Normal human cell proteins that interact with the adenovirus type 5 E1B 55kDa protein. Virology. 2017;504:12-24 pubmed publisher
    Several of the functions of the human adenovirus type 5 E1B 55kDa protein are fulfilled via the virus-specific E3 ubiquitin ligase it forms with the viral E4 Orf6 protein and several cellular proteins...
  47. Chen B, Weng Y, Shibu M, Han C, Chen Y, Shen C, et al. Estrogen and/or Estrogen Receptor ? Inhibits BNIP3-Induced Apoptosis and Autophagy in H9c2 Cardiomyoblast Cells. Int J Mol Sci. 2018;19: pubmed publisher
    ..However, autophagy can lead to substantial cell death under certain circumstances. BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3), a hypoxia-induced marker, has been shown to induce both autophagy and ..
  48. Müncheberg S, Hay R, Ip W, Meyer T, Weis C, Brenke J, et al. E1B-55K-Mediated Regulation of RNF4 SUMO-Targeted Ubiquitin Ligase Promotes Human Adenovirus Gene Expression. J Virol. 2018;92: pubmed publisher
    Human adenovirus (HAdV) E1B-55K is a multifunctional regulator of productive viral replication and oncogenic transformation in nonpermissive mammalian cells...
  49. Yamanaka M, Tada Y, Kawamura K, Li Q, Okamoto S, Chai K, et al. E1B-55 kDa-defective adenoviruses activate p53 in mesothelioma and enhance cytotoxicity of anticancer agents. J Thorac Oncol. 2012;7:1850-1857 pubmed publisher
    ..We examined possible antitumor effects of adenoviruses with a deletion of the E1B-55kD gene (Ad-delE1B55) on mesothelioma and investigated combinatory actions with the first-line chemotherapeutic ..
  50. Lu P, Kamboj A, Gibson S, Anderson C. Poly(ADP-ribose) polymerase-1 causes mitochondrial damage and neuron death mediated by Bnip3. J Neurosci. 2014;34:15975-87 pubmed publisher
    ..Bcl-2/adenovirus E1B 19 kDa-interacting protein (Bnip3) is a prodeath BH3-only Bcl-2 protein family member that is induced in hypoxia, ..
  51. Dehart C, Perlman D, Flint S. Impact of the adenoviral E4 Orf3 protein on the activity and posttranslational modification of p53. J Virol. 2015;89:3209-20 pubmed publisher
    ..established that the p53 populations that accumulate in normal human cells exposed to etoposide or infected by an E1B 55-kDa protein-null mutant of human adenovirus type 5 carry a large number of posttranslational modifications at ..
  52. Liu G, Zou H, Luo T, Long M, Bian J, Liu X, et al. Caspase-Dependent and Caspase-Independent Pathways Are Involved in Cadmium-Induced Apoptosis in Primary Rat Proximal Tubular Cell Culture. PLoS ONE. 2016;11:e0166823 pubmed publisher
    ..Z-VAD-FMK prevented caspase-3 activation and apoptosis and decreased BNIP-3 (Bcl-2/adenovirus E1B 19-kDa interacting protein 3) expression levels and apoptosis-inducing factor/endonuclease G (AIF/Endo G) ..
  53. Gupta D, Bijarnia Mahay S, Saxena R, Kohli S, Dua Puri R, Verma J, et al. Identification of mutations, genotype-phenotype correlation and prenatal diagnosis of maple syrup urine disease in Indian patients. Eur J Med Genet. 2015;58:471-8 pubmed publisher
    Maple syrup urine disease (MSUD) is caused by mutations in genes BCKDHA, BCKDHB, DBT encoding E1α, E1β, and E2 subunits of enzyme complex, branched-chain alpha-ketoacid dehydrogenase (BCKDH)...
  54. Sassone F, Margulets V, Maraschi A, Rodighiero S, Passafaro M, Silani V, et al. Bcl-2/adenovirus E1B 19-kDa interacting protein (BNip3) has a key role in the mitochondrial dysfunction induced by mutant huntingtin. Hum Mol Genet. 2015;24:6530-9 pubmed publisher
    ..These results provide new compelling evidence that underlies the molecular mechanisms by which mutant htt causes mitochondrial dysfunction and cell death, suggesting BNip3 as a potential target for HD therapy. ..
  55. Zhang T, Xue L, Li L, Tang C, Wan Z, Wang R, et al. BNIP3 Protein Suppresses PINK1 Kinase Proteolytic Cleavage to Promote Mitophagy. J Biol Chem. 2016;291:21616-21629 pubmed
    ..Here, we demonstrate that BCL2/adenovirus E1B 19-kDa interacting protein 3 (BNIP3), a mitochondrial BH3-only protein, interacts with PINK1 to promote the ..
  56. Speiseder T, Hofmann Sieber H, Rodriguez E, Schellenberg A, Akyüz N, Dierlamm J, et al. Efficient Transformation of Primary Human Mesenchymal Stromal Cells by Adenovirus Early Region 1 Oncogenes. J Virol. 2017;91: pubmed publisher
    ..observations that human amniotic fluid cells (AFC) can be transformed by human adenovirus type 5 (HAdV-5) E1A/E1B oncogenes prompted us to identify the target cells in the AFC population that are susceptible to transformation...
  57. Pan S, Sharma P, Shah S, Deshpande D. Bitter taste receptor agonists alter mitochondrial function and induce autophagy in airway smooth muscle cells. Am J Physiol Lung Cell Mol Physiol. 2017;313:L154-L165 pubmed publisher
    ..Furthermore, the expression of mitochondrial protein BCL2/adenovirus E1B 19-kDa protein-interacting protein 3 (Bnip3) and mitochondrial localization of DLP1 were significantly upregulated ..
  58. Frank B, Marcu A, de Oliveira Almeida Petersen A, Weber H, Stigloher C, Mottram J, et al. Autophagic digestion of Leishmania major by host macrophages is associated with differential expression of BNIP3, CTSE, and the miRNAs miR-101c, miR-129, and miR-210. Parasit Vectors. 2015;8:404 pubmed publisher
    ..The protein expression levels of autophagy related 5 (ATG5), BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3), cathepsin E (CTSE), mechanistic target of rapamycin (MTOR), ..
  59. Berscheminski J, Brun J, Speiseder T, Wimmer P, Ip W, Terzic M, et al. Sp100A is a tumor suppressor that activates p53-dependent transcription and counteracts E1A/E1B-55K-mediated transformation. Oncogene. 2016;35:3178-89 pubmed publisher
    ..used as a model system to investigate tumorigenic processes in mammalian cells where the viral oncoproteins E1A and E1B-55K are absolutely required for oncogenic transformation, because they simultaneously accelerate cell cycle ..
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    ..that HAdV induces KAP1 deSUMOylation to minimize epigenetic gene silencing and to promote SUMO modification of E1B-55K by a so far unknown mechanism...
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    ..to measure expression of autophagy related proteins [beclin-1, light chain (LC) 3A, LC3B, p62, and BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3)] and ROS related proteins [catalase, thioredoxinreductase, glutathione ..
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    ..AFP) promoter integrated with hypoxia response element (HRE) to control the adenovirus early region 1b (E1B). Then the novel double-regulated adenovirus Ad-hTERT-HREAF (named SG505) was engineered...
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    ..Classic form of MSUD (CMSUD) is caused by mutations in BCKDHA, BCKDHB, DBT genes mostly. In this study, we analyzed the clinical and genetic characteristics of two patients with CMSUD...
  64. Hendgen Cotta U, Esfeld S, Rudi K, Miinalainen I, Klare J, Rassaf T. Cytosolic BNIP3 Dimer Interacts with Mitochondrial BAX Forming Heterodimers in the Mitochondrial Outer Membrane under Basal Conditions. Int J Mol Sci. 2017;18: pubmed publisher
    ..The B-cell lymphoma (BCL-2) family member BCL-2 adenovirus E1B 19 kDa-interacting protein 3 (BNIP3) is linked to mitochondrial targeting after homodimerization, where it ..
  65. Krömmelbein N, Wiebusch L, Schiedner G, Büscher N, Sauer C, Florin L, et al. Adenovirus E1A/E1B Transformed Amniotic Fluid Cells Support Human Cytomegalovirus Replication. Viruses. 2016;8: pubmed publisher
    ..Here, we provide first-time evidence that a permanent cell line immortalized by adenovirus type 5 E1A and E1B (CAP) is supporting the full HCMV replication cycle and is releasing infectious progeny...
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    ..We reveal temporally distinct responses to adenovirus genomes: a critical MRN-ATM DDR that must be inactivated by E1B-55K/E4-ORF3 viral oncoproteins and a global MRN-independent ATM DDR to viral nuclear domains that does not impact ..
  67. Ma X, Lin W, Lin Z, Hao M, Gao X, Zhang Y, et al. Liraglutide alleviates H2O2-induced retinal ganglion cells injury by inhibiting autophagy through mitochondrial pathways. Peptides. 2017;92:1-8 pubmed publisher
    ..The axon marker GAP43, autophagy and mitophagy indicators LC3A/B, Beclin-1, p62, Parkin, BCL2/Adenovirus E1B 19kDa protein-interacting protein 3-like (BNIP3L) and the key regulator of mitochondrial biogenesis PGC-1α were ..
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    ..24-nucleotide deletion within the CR2 region under control of the human telomerase reverse transcriptase promoter, E1b gene directed by the hypoxia response element, and VSTM1 gene controlled by the cytomegalovirus promoter...
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    To explore the expression of NF-E1b in colorectal cancer tissues and its association with various clinicopathological parameters and prognosis of the patients...
  70. Diao H, Liu B, Shi Y, Song C, Guo Z, Liu N, et al. MicroRNA-210 alleviates oxidative stress-associated cardiomyocyte apoptosis by regulating BNIP3. Biosci Biotechnol Biochem. 2017;81:1712-1720 pubmed publisher
    ..The expression of miR-210 was upregulated in myocardial tissues of I/R rats, while that of Bcl-2 adenovirus E1B 19kDa-interacting protein 3 (BNIP3) was downregulated...
  71. Li X, Yang Y, Gao Q, Gao M, Lv Y, Dong R, et al. Clinical characteristics and mutation analysis of five Chinese patients with maple syrup urine disease. Metab Brain Dis. 2018;33:741-751 pubmed publisher
    ..Presently, at least 4 genes of BCKDHA, BCKDHB, DLD and DBT have been reported to cause MSUD...
  72. Alshudukhi A, Zhu J, Huang D, Jama A, Smith J, Wang Q, et al. Lipin-1 regulates Bnip3-mediated mitophagy in glycolytic muscle. FASEB J. 2018;:fj201800374 pubmed publisher
    ..studies using lipin-1-deficient myoblasts suggest that lipin-1 participates in B-cell leukemia (BCL)-2 adenovirus E1B 19 kDa protein-interacting protein 3 (Bnip3)-regulated mitophagy by interacting with microtubule-associated protein ..
  73. Diehl Jones W, Archibald A, Gordon J, Mughal W, Hossain Z, Friel J. Human Milk Fortification Increases Bnip3 Expression Associated With Intestinal Cell Death In Vitro. J Pediatr Gastroenterol Nutr. 2015;61:583-90 pubmed publisher
    ..HMF increases the expression of BCL2/adenovirus E1B 19 kDa protein-interacting protein (Bnip3) and cell death; the prostaglandin analogue misoprostol will rescue this ..
  74. Fordjour P, Wang L, Gao H, Li L, Wang Y, Nyagblordzro M, et al. Targeting BNIP3 in inflammation-mediated heart failure: a novel concept in heart failure therapy. Heart Fail Rev. 2016;21:489-97 pubmed publisher
    Myocardial injury activates inflammatory mediators and provokes the integration of BCL-2/adenovirus E1B 19KD interacting protein 3 (BNIP3) into mitochondrial membranes...
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    ..However, the mechanism of selective replication of E1b-deleted Ads in cancer cells is still not well characterized...
  76. Shen Y, Yang F, Wang H, Cai Z, Xu Y, Zhao A, et al. Arg-Gly-Asp (RGD)-Modified E1A/E1B Double Mutant Adenovirus Enhances Antitumor Activity in Prostate Cancer Cells In Vitro and in Mice. PLoS ONE. 2016;11:e0147173 pubmed publisher
    ..CAR mediates adenoviral infection, as well as adenovirus-mediated oncolysis of AxdAdB-3, an E1A/E1B double-restricted oncolytic adenovirus, in prostate cancer cells...
  77. Radke J, Yong S, Cook J. Low-Level Expression of the E1B 20-Kilodalton Protein by Adenovirus 14p1 Enhances Viral Immunopathogenesis. J Virol. 2016;90:497-505 pubmed publisher
    ..We found that human cell infection with Ad14p1 results in markedly decreased expression of the E1B 20-kilodalton (20K) protein compared to that with infection with wild-type (wt) Ad14...
  78. Choi J, Jo A, Kim M, Park H, Chung S, Kang S, et al. BNIP3 is essential for mitochondrial bioenergetics during adipocyte remodelling in mice. Diabetologia. 2016;59:571-81 pubmed publisher
    ..BCL2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3) is a mitochondrial protein and a known mitochondrial quality ..
  79. Koncsos G, Varga Z, Baranyai T, Boengler K, Rohrbach S, Li L, et al. Diastolic dysfunction in prediabetic male rats: Role of mitochondrial oxidative stress. Am J Physiol Heart Circ Physiol. 2016;311:H927-H943 pubmed publisher
    ..of mitofusin-2 was increased, while the phosphorylation of phospholamban and expression of Bcl-2/adenovirus E1B 19-kDa protein-interacting protein 3 (BNIP3, a marker of mitophagy) decreased...
  80. Li T, Wang Y, Li C, Xu W, Niu F, Zhang D. [Maple syrup urine disease and gene mutations in twin neonates]. Zhongguo Dang Dai Er Ke Za Zhi. 2016;18:1242-1246 pubmed
    ..blood samples were collected from the twin neonates and their parents to detect the genes related to MSUD (BCKDHA, BCKDHB, DBT, and DLD). The loci with gene mutations were identified, and a bioinformatic analysis was performed...
  81. Razavi Azarkhiavi K, Jaafari M, Abnous K, Razavi B, Jafarian A, Hassani F, et al. The Cardiotoxic Mechanism of Doxorubicin (DOX) and Pegylated Liposomal DOX in Mice Bearing C-26 Colon Carcinoma: a Study Focused on microRNA Role for Toxicity Assessment of New Formulations. Pharm Res. 2017;34:1849-1856 pubmed publisher
    ..investigate the expression of cardiac caspase-3, -8, Bax, Bcl2, Programmed cell death 4 (PDCD4) and BCL2/Adenovirus E1B 19 kDa Interacting Protein 3 (BNIP3)...
  82. Aevarsson A, Chuang J, Wynn R, Turley S, Chuang D, Hol W. Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. Structure. 2000;8:277-91 pubmed
    ..in MSUD patients, we determined the crystal structure of branched-chain alpha-ketoacid dehydrogenase (E1), the 170 kDa alpha(2)beta(2) heterotetrameric E1b component of the branched-chain alpha-ketoacid dehydrogenase multienzyme complex.
  83. Wynn R, Machius M, Chuang J, Li J, Tomchick D, Chuang D. Roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site. J Biol Chem. 2003;278:43402-10 pubmed
    ..of either His291-alpha or His146-beta' in the active site of human branched-chain alpha-ketoacid dehydrogenase (E1b) impede both the decarboxylation and the reductive acylation reactions catalyzed by E1b as well as the binding of ..
  84. Zong Y, Zhou S, Fatima S, Sorrentino B. Expression of mouse Abcg2 mRNA during hematopoiesis is regulated by alternative use of multiple leader exons and promoters. J Biol Chem. 2006;281:29625-32 pubmed
    ..We have recently identified three novel leader exons (termed E1A, E1B, and E1C) located in the 5'-untranslated region of mouse Abcg2 mRNA by data base searches and reverse transcription-..
  85. Wang Y, Qi M, Li T, Zhao Y. Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD). Gene. 2012;498:112-5 pubmed publisher
    ..BCKD is a mitochondrial complex encoded by four nuclear genes (BCKDHA, BCKDHB, DBT, and DLD) and is involved in the metabolism of branched-chain amino acids (BCAAs)...
  86. Xie Y, Natarajan K, Bauer K, Nakanishi T, Beck W, Moreci R, et al. Bcrp1 transcription in mouse testis is controlled by a promoter upstream of a novel first exon (E1U) regulated by steroidogenic factor-1. Biochim Biophys Acta. 2013;1829:1288-99 pubmed publisher
    ..alternative promoters associated with mRNAs containing alternative untranslated first exons designated as E1A, E1B, and E1C. The E1B promoter regulates Bcrp1 transcription in mouse intestine...
  87. Wu A, Yang M, Dalvi P, Turinsky A, Wang W, Butcher D, et al. Role of STAT5 and epigenetics in lactation-associated upregulation of multidrug transporter ABCG2 in the mammary gland. Am J Physiol Endocrinol Metab. 2014;307:E596-610 pubmed publisher
    ..Expression profiling of different mouse Abcg2 mRNA isoforms (E1a, E1b, and E1c) revealed that E1b is predominantly expressed and induced in the lactating mouse mammary gland...
  88. Stojiljkovic M, Klaassen K, Djordjevic M, Sarajlija A, Brasil S, Kecman B, et al. Molecular and phenotypic characteristics of seven novel mutations causing branched-chain organic acidurias. Clin Genet. 2016;90:252-7 pubmed publisher
    ..Thr198Serfs*6 in MMAA, p.Ile144_Leu181del in PCCB, p.Gly288Valfs*11, p.Tyr438Asn in BCKDHA and p.Ala137Val in BCKDHB gene. Interestingly, we identified seven novel genetic variants: p.Leu549Pro, p.Glu564*, p.Leu641Pro in MUT, p...
  89. Sándor S, Jordanidisz T, Schamberger A, Várady G, Erdei Z, Apáti A, et al. Functional characterization of the ABCG2 5' non-coding exon variants: Stem cell specificity, translation efficiency and the influence of drug selection. Biochim Biophys Acta. 2016;1859:943-51 pubmed publisher
    ..Although certain variants (E1B and E1C) are considered as "constitutive" mRNA isoforms, we show that chemotoxic drugs significantly alter the ..
  90. Chiu H, Yeh Y, Wang Y, Huang W, Ho S, Lin P, et al. Combination of the novel histone deacetylase inhibitor YCW1 and radiation induces autophagic cell death through the downregulation of BNIP3 in triple-negative breast cancer cells in vitro and in an orthotopic mouse model. Mol Cancer. 2016;15:46 pubmed publisher
    ..Bcl-2/adenovirus E1B 19 kDa protein-interacting protein 3 (BNIP3) is a member of the Bcl-2 subfamily...
  91. Rai A, Singh A, Saxena A, Seth T, Raina V, Mitra D. Exonal switch down-regulates the expression of CD5 on blasts of acute T cell leukaemia. Clin Exp Immunol. 2017;190:340-350 pubmed publisher
    ..Thirty-nine patients were found to be of ALL-T origin. Amplification of early region of E1A and E1B transcripts of CD5 was correlated with the levels of surface and intracellular expression of CD5 protein...