Rabbit anti-Human PDHX Polyclonal Antibody | anti-PDHX antibody

PDHX Antibody - middle region

Cat. Num. AAA3221857

• Gene Names: PDHX; E3BP; OPDX; PDX1; proX; DLDBP; PDHXD
• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity purified
• Synonyms: PDHX; Polyclonal Antibody; PDHX Antibody - middle region; anti-PDHX antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Affinity purified
• Form/Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Sequence: Synthetic peptide located within the following region: GSKNIRLGSLIGLIVEEGEDWKHVEIPKDVGPPPPVSKPSEPRPSPEPQI
• Sequence Length: 501

• Applicable Applications for anti-PDHX antibody: Western Blot (WB)
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human PDHX
• Preparation and Storage: For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Host: RabbitTarget Name: PDHXSample Tissue: Human Mesenchymoma Tumor lysatesAntibody Dilution: 1ug/ml)

Related Product Information for anti-PDHX antibody

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor 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 eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Product Categories/Family for anti-PDHX antibody

Polyclonal; Cell Biology; Mitochondria; E3 & Ubiquitin; Disease Related

NCBI and Uniprot Product Information

• NCBI GI #: 203098816
• NCBI GeneID: 8050
• NCBI Accession #: NP_001128496.1
• NCBI GenBank Nucleotide #: NM_001135024.1
• UniProt Accession #: O00330
• Molecular Weight: 55 kDa
• NCBI Official Full Name: pyruvate dehydrogenase protein X component, mitochondrial isoform 2
• NCBI Official Synonym Full Names: pyruvate dehydrogenase complex component X
• NCBI Official Symbol: PDHX
• NCBI Official Synonym Symbols: E3BP; OPDX; PDX1; proX; DLDBP; PDHXD
• NCBI Protein Information: pyruvate dehydrogenase protein X component, mitochondrial
• UniProt Protein Name: Pyruvate dehydrogenase protein X component, mitochondrial
• Protein Family: Pyruvate dehydrogenase complex
• UniProt Gene Name: PDHX
• UniProt Synonym Gene Names: PDX1; E3BP
• UniProt Entry Name: ODPX_HUMAN

NCBI Description

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor 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 eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]

Uniprot Description

PDHX: a mitochondrial protein required for anchoring dihydrolipoamide dehydrogenase (E3) to the pyruvate dehydrogenase complexes (PDHC) of eukaryotes. This specific binding is essential for a PDHC function. Eukaryotic pyruvate dehydrogenase complexes are organized about a core consisting of the oligomeric dihydrolipoamide acetyl-transferase (E2), around which are arranged multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide dehydrogenase (E3), and protein X bound by non-covalent bonds. Belongs to the 2-oxoacid dehydrogenase family. Contains 1 lipoyl-binding domain.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 11p13

Cellular Component: mitochondrial matrix

Molecular Function: transferase activity, transferring acyl groups

Biological Process: cellular metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E3-binding Protein Deficiency