免疫原
Synthesized peptide derived from ACAD-9 . at AA range: 530-610
特异性
ACAD-9 Polyclonal Antibody detects endogenous levels of ACAD-9 protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
This gene encodes a member of the acyl-CoA dehydrogenase family. Members of this family of proteins localize to the mitochondria and catalyze the rate-limiting step in the beta-oxidation of fatty acyl-CoA. The encoded protein is specifically active toward palmitoyl-CoA and long-chain unsaturated substrates. Mutations in this gene cause acyl-CoA dehydrogenase family member type 9 deficiency. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Mar 2010],
功能
cofactor:FAD.,disease:Defects in ACAD9 are a cause of acyl-CoA dehydrogenase family member type 9 deficiency (ACAD9 deficiency) [MIM:611126]. ACAD9 deficiency patients present with episodic liver dysfunction during otherwise mild illnesses or cardiomyopathy, along with chronic neurologic dysfunction.,function:Has a dehydrogenase activity on palmitoyl-CoA (C16:0) and stearoyl-CoA (C18:0). It is three times more active on palmitoyl-CoA then on stearoyl-CoA. Has little activity on octanoyl-CoA (C8:0), butyryl-CoA (C4:0) or isovaleryl-CoA (5:0).,similarity:Belongs to the acyl-CoA dehydrogenase family.,tissue specificity:Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver.,
蛋白名称
Acyl-CoA dehydrogenase family member 9 mitochondrial
其他名称
ACAD9; Acyl-CoA dehydrogenase family member 9; mitochondrial; ACAD-9
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side . Essentially associated with membranes. .
组织表达
Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver (PubMed:12359260). In the cerebellum uniquely expressed in the granular layer (at protein level) (PubMed:21237683).
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