Product Name	PCB
Chinese Name	丙酮酸羧化酶抗体
Alias	mitochondrial; OTTHUMP00000235155; OTTHUMP00000235156; PC; PCB; Pcx; PYC_HUMAN; Pyruvate carboxylase; Pyruvate carboxylase mitochondrial; Pyruvic carboxylase.
Research Area	Tumour  Cell biology  immunology  Signal transduction  The new supersedes the old
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse,  (predicted: Rat, bovine)
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	127kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PCB : 21-120/1178
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	PCB (Pyruvate carboxylase) requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. PCB is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency. Alternatively spliced transcript variants with different 5' UTRs, but encoding the same protein, have been found for this gene. Function: Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. Subunit: Homotetramer. Subcellular Location: Mitochondrion matrix. DISEASE: Pyruvate carboxylase deficiency (PC deficiency) [MIM:266150]: Leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Contains 1 ATP-grasp domain. Contains 1 biotin carboxylation domain. Contains 1 biotinyl-binding domain. Contains 1 carboxyltransferase domain. SWISS: P11498 Gene ID: 5091 Database links: UniProtKB/Swiss-Prot: P11498.2
Product Picture	Sample: Lane 1: Mouse Liver tissue lysates Primary: Anti-PCB (SL21700R) at 1/1000 dilution Secondary: Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution Predicted band size: 127 kDa Observed band size: 127 kDa Sample: Lane 1: Human HepG2 cell lysates Primary: Anti-PCB (SL21700R) at 1/1000 dilution Secondary: Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution Predicted band size: 127 kDa Observed band size: 127 kDa Paraformaldehyde-fixed, paraffin embedded (human pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (PCB) Polyclonal Antibody, Unconjugated (SL21700R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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