PDHX Knockout cell line (HEK293)

Catalog Number: KO06535

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Specifications

Product Information
Product Name	PDHX Knockout cell line (HEK293)
specification	1*10^6
Storage and transportation	Dry ice preservation/T25 live cell transportation.
Cell morphology	Epithelioid, adherent cell
Passage ratio	1:3~1:6
species	Human
Gene	PDHX
Gene ID	8050
Build method	Electric rotation method / virus method
Mycoplasma testing	Negative
Cultivation system	90%DMEM+10% FBS
Parental Cell Line	HEK293
Quality Control	Genotype: PDHX Knockout cell line (HEK293) >95% viability before freezing. All cells were tested and found to be free of bacterial, viruses,mycoplasma and other toxins.

Gene Information
Gene Official Full Name	pyruvate dehydrogenase complex component Xprovided by HGNC
Also known as	E3BP; OPDX; PDX1; proX; DLDBP; PDHXD
Gene 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]
Expression	Ubiquitous expression in heart (RPKM 24.7), testis (RPKM 13.6) and 25 other tissues See more

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