异戊酰辅酶A脱氢酶抗体

规格：1mg/1ml

英文名： IVD

别名： ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial.

分子量： 43kDa

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

免疫原：KLH conjugated synthetic peptide derived from human IVD

交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,

细胞定位：

异戊酰辅酶A脱氢酶抗体产品介绍：background: Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009] Function: Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious异戊酰辅酶A脱氢酶抗体 vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Similarity: Belongs to the acyl-CoA dehydrogenase family. Gene ID: 3712 Database links: Entrez Gene: 3712 Human 异戊酰辅酶A脱氢酶抗体Entrez Gene: 510440 Cow Entrez Gene: 56357 Mouse Entrez Gene: 100156047 Pig Entrez Gene: 24513 Rat Omim: 607036 Human SwissProt: Q3SZI8 Cow SwissProt: P26440 Human SwissProt: Q9JHI5 Mouse SwissProt: P12007 Rat Unigene: 513646 Human Unigene: 6635 Mouse Unigene: 147 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

异戊酰辅酶A脱氢酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=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  神经生物学  信号转导  新陈代谢  干扰素  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid