Metabolism Antibodies 1

Anti-DLAT Antibody (CAB14530)

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SKU:
CAB14530
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-DLAT Antibody
Antibody SKU:CAB14530
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 87-270 of human DLAT (NP_001922.2).
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HeLa, MCF7, HepG2, K-562, Mouse brain, Rat brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 87-270 of human DLAT (NP_001922.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:SLPP HQKV PLPS LSPT MQAG TIAR WEKK EGDK INEG DLIA EVET DKAT VGFE SLEE CYMA KILV AEGT RDVP IGAI ICIT VGKP EDIE AFKN YTLD SSAA PTPQ AAPA PTPA ATAS PPTP SAQA PGSS YPPH MQVL LPAL SPTM TMGT VQRW EKKV GEKL SEGD LLAE IETD KATI GFEV QEEG
Gene ID:1737
Uniprot:P10515
Cellular Location:Mitochondrion matrix
Calculated MW:68kDa
Observed MW:69kDa
Synonyms:DLAT, DLTA, PDC-E2, PDCE2
Background: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.
UniProt Protein Function:DLAT: the E2 component of the pyruvate dehydrogenase complex (PDHC) that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. Contains two lipoyl-binding domains. The PDHC plays a major role in controlling the balance between lipid and glucose oxidation depending on substrate availability. The activity of the PDHC is tightly regulated by phosphorylation of the E1 components by the PDHKs. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homo-dimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. PDHKs are recruited to the PDHC by binding to a lipoyl group covalently attached to K174 of the inner lipoyl domain of the E2 component.
UniProt Protein Details:

Protein type:Mitochondrial; EC 2.3.1.12; Transferase; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - glycolysis and gluconeogenesis

Chromosomal Location of Human Ortholog: 11q23.1

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial pyruvate dehydrogenase complex

Molecular Function:dihydrolipoyllysine-residue acetyltransferase activity; protein binding

Biological Process: cellular metabolic process; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E2 Deficiency

NCBI Summary: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]
UniProt Code:P10515
NCBI GenInfo Identifier:215274207
NCBI Gene ID:1737
NCBI Accession:P10515.3
UniProt Secondary Accession:P10515,Q16783, Q53EP3,
UniProt Related Accession:P10515
Molecular Weight:647
NCBI Full Name:Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
NCBI Synonym Full Names:dihydrolipoamide S-acetyltransferase
NCBI Official Symbol:DLAT  
NCBI Official Synonym Symbols:DLTA; PDCE2; PDC-E2  
NCBI Protein Information:dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; PBC; M2 antigen complex 70 kDa subunit; pyruvate dehydrogenase complex component E2; E2 component of pyruvate dehydrogenase complex; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
UniProt Protein Name:Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
UniProt Synonym Protein Names:70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; Pyruvate dehydrogenase complex component E2; PDC-E2; PDCE2
Protein Family:Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex
UniProt Gene Name:DLAT  
UniProt Entry Name:ODP2_HUMAN
Anti-DLAT Antibody (CAB14530)Western blot analysis of extracts of various cell lines, using DLAT antibody (CAB14530) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
Anti-DLAT Antibody (CAB14530)Immunohistochemistry of paraffin-embedded rat kidney using DLAT antibody (CAB14530) at dilution of 1:100 (40x lens).
Anti-DLAT Antibody (CAB14530)Immunohistochemistry of paraffin-embedded human colon carcinoma using DLAT antibody (CAB14530) at dilution of 1:100 (40x lens).
Anti-DLAT Antibody (CAB14530)Immunohistochemistry of paraffin-embedded mouse kidney using DLAT antibody (CAB14530) at dilution of 1:100 (40x lens).
Anti-DLAT Antibody (CAB14530)Immunofluorescence analysis of L929 cells using DLAT antibody (CAB14530) at dilution of 1:100. Blue: DAPI for nuclear staining.
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