Catalog Number:
E20-70041Amount:
100ulMolecular Weight:
102132DaApplications:
WB;ELISAReactivity:
Human;Rat;Mouse;Purification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.Immunogen:
The antiserum was produced against synthesized peptide derived from human AASS. AA range:251-300Source:
Polyclonal, Rabbit,IgG
Gene Name:
AASSDilution:
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.Background:
This gene encodes a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial hyperlysinemia. [provided by RefSeq, Jul 2008],Protein Name:
Alpha-aminoadipic semialdehyde synthase mitochondrialHuman Gene Id:
10157Human Swiss Prot No:
Q9UDR5Mouse Swiss Prot No:
Q99K67Specificity:
AASS Polyclonal Antibody detects endogenous levels of AASS protein.Formulation:
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.Concentration:
1 mg/mlStorage Stability:
-20°C/1 yearOther Name:
AASS; Alpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDHObserved Band(KD):
102Function:
catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + 2-aminoadipate 6-semialdehyde + NADH.,catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O = L-lysine + 2-oxoglutarate + NADPH.,disease:Defects in AASS are the cause of hyperlysinemia [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.,function:Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.,induction:Induced by starvation.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysSubcellular Location:
Mitochondrion .Expression:
Expressed in all 16 tissues examined with highest expression in the liver.Catalog Number:
E011009-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
145$
Catalog Number:
E011009-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
225$
Catalog Number:
E011013-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
145$
Catalog Number:
E011013-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
225$
Catalog Number:
E011020-1Amount:
50μg/50μlReactivity:
HumanApplications:
IF:1:100~1:200Swiss-Prot No.:
Q04864Price:
145$