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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 15 Apr 2015.

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, CAN, HAD, Phosphogluconate Dehydrogenase, POLYMERASE
Papers on PBC
Addition of ethylene to a π-conjugated two-dimensional nickel-based organometallic framework with implications for olefin separation.
New
Hall et al., Doha, Qatar. In J Mol Model, 31 May 2015
We considered periodic boundary calculations (PBC) as well as clusters containing up to 12 nickel atoms using a screened hybrid density functional to obtain accurate reaction barriers.
The Biliary Epithelium Presents Antigens to and Activates Natural Killer T Cells.
New
Melum et al., Oslo, Norway. In Hepatology, 08 May 2015
CD1d expression was down-regulated in the biliary epithelium of patients with late primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC) and alcoholic cirrhosis compared to healthy controls.
A poly(lactide-co-glycolide) film loaded with abundant bone morphogenetic protein-2: A substrate-promoting osteoblast attachment, proliferation, and differentiation in bone tissue engineering.
New
Kang et al., Chongqing, China. In J Biomed Mater Res A, 03 May 2015
Using phospholipid as a surfactant, BMP-2 was modified as a complex (PBC) for dispersing in PLGA/dichloromethane solution.
Synthesis of diastereomerically pure Lys(N(ε) -lipoyl) building blocks and their use in Fmoc/tBu solid phase synthesis of lipoyl-containing peptides for diagnosis of primary biliary cirrhosis.
New
Papini et al., Cergy-Pontoise, France. In J Pept Sci, 26 Apr 2015
UNASSIGNED: Primary Biliary Cirrhosis is an immune-mediated disease in which one of the epitopes recognized by antimitochondrial autoantibodies is a lipoylated fragment of the PDC-E2 protein.
Recent advances in the development of farnesoid X receptor agonists.
Review
New
Lindor et al., Scottsdale, United States. In Ann Transl Med, Jan 2015
In two clinical trials of OCA in patients with primary biliary cirrhosis (PBC), a progressive cholestatic liver disease, OCA significantly reduced serum alkaline phosphatase (ALP) levels, an important disease marker that correlates well with clinical outcomes of patients with PBC.
Innate immunity drives the initiation of a murine model of primary biliary cirrhosis.
New
Chuang et al., Taipei, Taiwan. In Plos One, Dec 2014
Our earlier work suggested that iNKT cells were involved in the initiation of the original loss of tolerance in primary biliary cirrhosis (PBC).
Signal Transducer and Activator of Transcription 4 in Liver Diseases.
Review
New
Wang et al., Beijing, China. In Int J Biol Sci, Dec 2014
STAT4 gene polymorphism has been shown to be associated with the antiviral response in chronic hepatitis C and drug-induced liver injury (DILI), primary biliary cirrhosis (PBC), HCV-associated liver fibrosis and in hepatocellular carcinoma (HCC).
Pregnancy in women with primary biliary cirrhosis.
Review
New
Wahlin et al., Ankara, Turkey. In Autoimmun Rev, Sep 2014
BACKGROUND & AIMS: Primary biliary cirrhosis (PBC) may present in all decades of life, also in childbearing age.
Therapeutic role of bile acids and nuclear receptor agonists in fibrosing cholangiopathies.
Review
Hofer et al., Vienna, Austria. In Dig Dis, 2013
Chronic inflammatory bile duct diseases such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) result in progressive fibrosis of the biliary tract and ultimately cirrhosis of the liver.
Recent advances on the mechanisms regulating cholangiocyte proliferation and the significance of the neuroendocrine regulation of cholangiocyte pathophysiology.
Review
Gaudio et al., Roma, Italy. In Ann Transl Med, 2013
Cholangiocytes play several key roles in the modification of ductal bile and are also the target cells in chronic cholestatic liver diseases (i.e., cholangiopathies) such as PSC, PBC, polycystic liver disease (PCLD) and cholangiocarcinoma (CCA).
The immunobiology and pathophysiology of primary biliary cirrhosis.
Review
Impact
Gershwin et al., Birmingham, United Kingdom. In Annu Rev Pathol, 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.
Brain [U-13 C]glucose metabolism in mice with decreased α-ketoglutarate dehydrogenase complex activity.
GeneRIF
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.
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.
GeneRIF
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].
GeneRIF
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.
GeneRIF
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.
GeneRIF
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.
Impact
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.
Impact
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.
Impact
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.
Impact
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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