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Dihydrolipoamide S-acetyltransferase

PBC, E-2, PDC-E2, E2B
This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009] (from NCBI)
Top mentioned proteins: ACID, HAD, CAN, Phosphogluconate Dehydrogenase, POLYMERASE
Papers on PBC
Primary biliary cirrhosis in 2014.
Mayo et al., Dallas, United States. In Curr Opin Gastroenterol, May 2014
PURPOSE OF REVIEW: Primary biliary cirrhosis (PBC) was first described in the 1950s as a clinical syndrome of progressive cholestatic liver disease resulting from chronic inflammatory destruction of the intrahepatic bile ducts.
Fibrate treatment for primary biliary cirrhosis.
Trauner et al., Vienna, Austria. In Curr Opin Gastroenterol, May 2014
PURPOSE OF REVIEW: Primary biliary cirrhosis (PBC) can lead to end-stage liver disease and death.
Pathophysiological analysis of primary biliary cirrhosis focusing on choline/phospholipid metabolism.
Nakamuta et al., Fukuoka, Japan. In Liver Int, Apr 2014
BACKGROUND & AIMS: Injury to biliary epithelial cells caused by disorders in bile composition may be the initial step in the pathogenesis of primary biliary cirrhosis (PBC).
The Arabidopsis thaliana FASCICLIN LIKE ARABINOGALACTAN PROTEIN 4 gene acts synergistically with abscisic acid signalling to control root growth.
Acet et al., Vienna, Austria. In Ann Bot, Apr 2014
Salt oversensitivity in At-fla4 is suppressed by the CYP707A inhibitor abscinazole E2B, and salt oversensitivity in At-fla4 roots is phenocopied by chemical inhibition of ABA biosynthesis.ConclusionsThe predicted lipid-anchored glycoprotein At-FLA4 positively regulates cell wall biosynthesis and root growth by modulating ABA signalling.
International Conference on Harmonisation; E2B(R3) Electronic Transmission of Individual Case Safety Reports; Data Elements and Message Specification; Appendix on Backwards and Forwards Compatibility; availability. Notice.
Food and Drug Administration, In Fed Regist, Mar 2014
The Food and Drug Administration (FDA) is announcing the availability of a guidance for industry entitled "E2B(R3) Electronic Transmission of Individual Case Safety Reports (ICSRs): Implementation Guide--Data Elements and Message Specification'' (the E2B(R3) implementation guidance) and an appendix to the guidance entitled "ICSRs: Appendix to the Implementation Guide--Backwards and Forwards Compatibility'' (the BFC appendix).
Expression of vascular endothelial growth factors and their receptors by hepatic progenitor cells in human liver diseases.
Gaudio et al., Roma, Italy. In Hepatobiliary Surg Nutr, Apr 2013
HPC activation has been involved in the progression of chronic parenchymal diseases (chronic viral hepatitis) and chronic biliary diseases (such as Primary Biliary Cirrhosis: PBC) and in the occurrence of intrahepatic cholangiocarcinoma.
Is there a role for cyclophilin inhibitors in the management of primary biliary cirrhosis?
Mason et al., Edmonton, Canada. In Viruses, Feb 2013
Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are poorly understood autoimmune liver diseases.
The immunobiology and pathophysiology of primary biliary cirrhosis.
Gershwin et al., Birmingham, United Kingdom. In Annu Rev Pathol, Feb 2013
Fifth, several mouse models of PBC highlight the importance of loss of tolerance to PDC-E2 as well as a critical role for the interleukin (IL)-12 signaling pathway.
Pleiotropic functions of bile acids mediated by the farnesoid X receptor.
Mikov et al., Novi Sad, Serbia. In Acta Gastroenterol Belg, 2012
During a phase II clinical trial, the administration of a semisynthetic BA derivative 6-ethyl-chenodeoxycholic acid (6-ECDCA) to patients with diabetes, non-alcoholic fatty liver disease (NAFLD) and primary biliary cirrhosis (PBC), led to encouraging results, despite side effects being observed in pre-clinical studies.
Role of AE2 for pHi regulation in biliary epithelial cells.
Medina et al., Pamplona, Spain. In Front Physiol, 2012
Early studies showed that AE2 gene expression is reduced in liver biopsies and blood mononuclear cells from patients with primary biliary cirrhosis (PBC), a disease characterized by chronic non-suppurative cholangitis associated with antimitochondrial antibodies (AMA) and other autoimmune phenomena.
Brain [U-13 C]glucose metabolism in mice with decreased α-ketoglutarate dehydrogenase complex activity.
Sonnewald et al., Trondheim, Norway. In J Neurosci Res, 2011
These results imply that diminished KGDHC activity has the potential to induce the reduction in glucose utilization that is seen in several neurodegenerative diseases.
[Pathogenesis of biliary tract injury in primary biliary cirrhosis].
Shimoda et al., In Nihon Rinsho Meneki Gakkai Kaishi, 2011
Primary biliary cirrhosis (PBC) is histologically characterized by chronic nonsuppurative destructive cholangitis (CNSDC) associated with destruction of small bile ducts.
Nuclear localization of pyruvate dehydrogenase complex-E2 (PDC-E2), a mitochondrial enzyme, and its role in signal transducer and activator of transcription 5 (STAT5)-dependent gene transcription.
Yu et al., North Chicago, United States. In Cell Signal, 2011
a novel function of pyruvate dehydrogenase complex E2 in the nucleus in up-regulating the transactivating ability of STAT5
[2-Oxoglutarate dehydrogenase complex and its multipoint control].
Strumiło et al., Białystok, Poland. In Postepy Biochem, 2010
2-oxoglutarate dehydrogenase complex (OGDHC), the key regulatory enzyme of Krebs cycle. [review]
Four novel mutations identified in Norwegian patients result in intermittent maple syrup urine disease when combined with the R301C mutation.
Eide et al., Oslo, Norway. In Mol Genet Metab, 2010
4 novel mutations in DBT gene resulting in intermittent maple syrup urine disease in 7 Norwegian patients; pathogenic effect of the mutations is depletion of cellular protein; intermittent form of MSUD appears to be due to residual R301C mutant protein
Solution structure and characterisation of the human pyruvate dehydrogenase complex core assembly.
Byron et al., Glasgow, United Kingdom. In J Mol Biol, 2010
Solution structure and characterisation of the human pyruvate dehydrogenase complex core assembly
Tumor transcriptome reveals the predictive and prognostic impact of lysosomal protease inhibitors in non-small-cell lung cancer.
Collie-Duguid et al., Aberdeen, United Kingdom. In J Clin Oncol, 2006
PURPOSE: Insight into clinical response to platinum-based chemotherapy (PBC) in non-small-cell lung cancer (NSCLC).
Hormone receptor status of a contralateral breast cancer is independent of the receptor status of the first primary in patients not receiving adjuvant tamoxifen.
Osborne et al., Houston, United States. In J Clin Oncol, 2005
PURPOSE: To determine whether the hormone receptor status of the primary breast cancer (PBC) is predictive of the hormone receptor status of the subsequent contralateral breast cancer (CBC).
5-HT4(a) receptors avert opioid-induced breathing depression without loss of analgesia.
Richter et al., Göttingen, Germany. In Science, 2003
This is caused by direct inhibition of rhythm-generating respiratory neurons in the Pre-Boetzinger complex (PBC) of the brainstem.
Phase I trial of recombinant fusion protein PIXY321 for mobilization of peripheral-blood cells.
Kessinger et al., Omaha, United States. In J Clin Oncol, 1996
PURPOSE: Mobilization of peripheral-blood cells (PBC) with cytokines alone results in rapid hematopoietic recovery and avoids the potential morbidity associated with mobilization by chemotherapy.
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