产品名称
ACAD-11 Rabbit Polyclonal Antibody
别名
ACAD11; Acyl-CoA dehydrogenase family member 11; ACAD-11
蛋白名称
Acyl-CoA dehydrogenase family member 11
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=84129
Human Swissprot No.
Q709F0
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q709F0/entry
Mouse Swissprot No.
Q80XL6
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q80XL6
免疫原
The antiserum was produced against synthesized peptide derived from human ACAD11. AA range:381-430
特异性
ACAD-11 Polyclonal Antibody detects endogenous levels of ACAD-11 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
acyl-CoA dehydrogenase family member 11(ACAD11) Homo sapiens This gene encodes an acyl-CoA dehydrogenase enzyme with a preference for carbon chain lengths between 20 and 26. Naturally occurring read-through transcription occurs between the upstream gene NPHP3 (nephronophthisis 3 (adolescent)) and this gene. [provided by RefSeq, Aug 2015],
组织表达
Widely expressed with highest levels in brain followed by liver, heart and kidney.
细胞定位
Peroxisome . Mitochondrion membrane . Has been detected associated with mitochondrial membrane, but no matrix, in kidney and cerebellum, as well as in a neuroblastoma cell line, but not in skin fibroblasts, where it is observed in cytoplasmic vesicles (PubMed:21237683). No mitochondrial targeting signals could be predicted for any known isoform, including a putative isoform starting at Met-316. .
功能
alternative products:Additional isoforms seem to exist,disease:Defects in NPHP3 are a cause of renal-hepatic-pancreatic dysplasia (RHPD) [MIM:208540]. RHPD is an autosomal recessive disorder with variable expression, and patients surviving the neonatal period progress to renal and hepatic failure which can be treated successfully with combined liver-kidney transplantation.,disease:Defects in NPHP3 are the cause of nephronophthisis type 3 (NPHP3) [MIM:604387]; also known as adolescent nephronophthisis. NPHP3 is a autosomal recessive disorder resulting in end-stage renal disease. It is characterized by polyuria, polydipsia, anemia. Onset of terminal renal failure occurr significantly later (median age, 19 years) than in juvenile nephronophthisis. Renal pathology is characterized by alterations of tubular basement membranes, tubular atrophy and dilatation, sclerosing tubulointerstitial nephropathy, and renal cyst development predominantly at the corticomedullary junction.,function:May participate in mechanosensation in the primary cilium of kidney cells.,similarity:Belongs to the acyl-CoA dehydrogenase family.,similarity:Contains 11 TPR repeats.,subunit:Interacts with NPHP1.,tissue specificity:Widely expressed at low level. Expressed in heart, placenta, liver, skeletal muscle, kidney and pancreas. Expressed at very low level in brain and lung.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
