Antibody Name:	Anti-PDHX Antibody
Antibody SKU:	CAB6426
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF IP
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).

Application:	WB IF IP
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:10 - 1:100 IP 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	SW620, HepG2, U-251MG, MCF7, Mouse heart, Mouse testis, Mouse brain, Rat kidney, Rat heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
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:	MAAS WRLG CDPR LLRY LVGF PGRR SVGL VKGA LGWS VSRG ANWR WFHS TQWL RGDP IKIL MPSL SPTM EEGN IVKW LKKE GEAV SAGD ALCE IETD KAVV TLDA SDDG ILAK IVVE EGSK NIRL GSLI GLIV EEGE DWKH VEIP KDVG PPPP VSKP SEPR PSPE PQIS IPVK KEHI PGTL RFRL SPAA RNIL EKHS LDAS QGTA TGPR GIFT KEDA LKLV QLKQ TGKI TESR PTPA PTAT PTAP SPLQ ATAG PSYP RPVI PPVS TPGQ PNAV GTFT EIPA SNIR RVIA KRLT ESKS TVPH
Gene ID:	8050
Uniprot:	O00330
Cellular Location:	Mitochondrion matrix
Calculated MW:	29kDa/51kDa/54kDa
Observed MW:	54kDa

Synonyms:

PDHX, DLDBP, E3BP, OPDX, PDX1, proX

Background:

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.

UniProt Protein Function:	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.
UniProt Protein Details:	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
NCBI Summary:	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 Code:	O00330
NCBI GenInfo Identifier:	12643417
NCBI Gene ID:	8050
NCBI Accession:	O00330.3
UniProt Related Accession:	O00330
Molecular Weight:
NCBI Full Name:	Pyruvate dehydrogenase protein X component, mitochondrial
NCBI 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
UniProt Synonym Protein Names:	Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex; E3-binding protein; E3BP; Lipoyl-containing pyruvate dehydrogenase complex component X; proX
Protein Family:	Pyruvate dehydrogenase complex
UniProt Gene Name:	PDHX
UniProt Entry Name:	ODPX_HUMAN

	Western blot analysis of extracts of various cell lines, using PDHX antibody (CAB6426) 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: 5s.
	Immunofluorescence analysis of MCF7 cells using PDHX antibody (CAB6426). Blue: DAPI for nuclear staining.
	Immunoprecipitation analysis of 600ug extracts of Mouse brain cells using 3ug PDHX antibody (CAB6426). Western blot was performed from the immunoprecipitate using PDHX antibody (CAB6426) at a dilition of 1:1000.