Molecular weight (DA)
102kD
Immunogen
The antiserum was produced against synthesized peptide derived from human AASS. AA range:251-300
Specificity
AASS Polyclonal Antibody detects endogenous levels of AASS protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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],
Function
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-lys
Protein name
Alpha-aminoadipic semialdehyde synthase mitochondrial
Other name
AASS; Alpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDH
Fields
>>Lysine degradation;>>Metabolic pathways
Human protein sequence Database
Q9UDR5
Mouse protein sequence database
Q99K67
Rat protein sequence database
Cellular localization
Mitochondrion .
Tissue expression
Expressed in all 16 tissues examined with highest expression in the liver.
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