PC Antibody | MyBioSource MBS853249 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

PC Antibody

catalog :

MBS853249

quantity :

0.1 mg

price :

215 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS853249

products type :

Antibody

products full name :

PC Antibody

products short name :

products name syn :

EC 6.4.1.1; PCB; PYC; Pyruvate carboxylase; mitochondrial; Pyruvic carboxylase

other names :

pyruvate carboxylase, mitochondrial; Pyruvate carboxylase, mitochondrial; pyruvate carboxylase, mitochondrial; pyruvic carboxylase; pyruvate carboxylase; Pyruvic carboxylase; PCB

products gene name :

other gene names :

PC; PC; PCB; PCB

uniprot entry name :

PYC_HUMAN

host :

Rabbit

reactivity :

Human, Mouse, Rat

sequence length :

1178

specificity :

PC antibody detects endogenous levels of total PC protein.

purity :

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

form :

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

storage stability :

Store at -20 degree C for 1 year

tested application :

Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)

app notes :

WB: 1:500~1:1000, IHC: 1:50~1:100, ELISA: 1:40000

other info1 :

Reactivity Note: Human (Identities = 100%, Positives = 100%); Mouse (Identities = 100%, Positives = 100%); Rat (Identities = 100%, Positives = 100%)

other info2 :

Immunogen: The antiserum was produced against synthesized peptide derived from internal of human PC. Pathway: Translation

products categories :

Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody

products references :

Wexler I.D., Biochim. Biophys. Acta 1227:46-52(1994). Mackay N., Biochem. Biophys. Res. Commun. 202:1009-1014(1994). Lamhonwah A.-M., Arch. Biochem. Biophys. 254:631-636(1987).

ncbi gi num :

106049295

ncbi acc num :

NP_000911.2

ncbi gb acc num :

NM_000920.3

uniprot acc num :

P11498

ncbi mol weight :

57,925 Da

ncbi pathways :

Alanine And Aspartate Metabolism Pathway (198783); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Biotin Transport And Metabolism Pathway (106257); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Citrate Cycle (TCA Cycle) Pathway (82927); Citrate Cycle (TCA Cycle) Pathway (288); Defective AMN Causes Hereditary Megaloblastic Anemia 1 Pathway (906000); Defective BTD Causes Biotidinase Deficiency Pathway (906015)

ncbi summary :

This gene encodes pyruvate carboxylase, which 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. Pyruvate carboxylase 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. [provided by RefSeq, Jul 2008]

uniprot summary :

PC: 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. Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency). PC deficiency 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. Protein type: Mitochondrial; Ligase; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - citrate (TCA) cycle; EC 6.4.1.1. Chromosomal Location of Human Ortholog: 11q13.4-q13.5. Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm; cytosol. Molecular Function: protein binding; pyruvate carboxylase activity; DNA binding; metal ion binding; biotin carboxylase activity; biotin binding; ATP binding. Biological Process: oxaloacetate metabolic process; vitamin metabolic process; carbohydrate metabolic process; viral RNA genome packaging; glucose metabolic process; pathogenesis; lipid metabolic process; pyruvate metabolic process; water-soluble vitamin metabolic process; biotin metabolic process; gluconeogenesis. Disease: Pyruvate Carboxylase Deficiency

size1 :

0.1 mg

price1 :

215 USD

more info or order :

MyBioSource product webpage