丙酮酸羧化酶抗体
规格：1mg/1ml
英文名： PCB
别名： mitochondrial; OTTHUMP00000235155; OTTHUMP00000235156; PC; PCB; Pcx; PYC_HUMAN; Pyruvate carboxylase; Pyruvate carboxylase mitochondrial; Pyruvic carboxylase.
分子量： 127kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PCB
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：
丙酮酸羧化酶抗体产品介绍：background: 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丙酮酸羧化酶抗体 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 丙酮酸羧化酶抗体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. Database links: UniProtKB/Swiss-Prot: P11498.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酮酸羧化酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid