BD-PT0041

AASS Rabbit Polyclonal Antibody

AASS; Alpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDH

常规抗体

AASS

Alpha-aminoadipic semialdehyde synthase mitochondrial

WB

Human,Rat,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

10157

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=10157

Q9UDR5

http://www.uniprot.org/uniprotkb/Q9UDR5/entry

Q99K67

http://www.uniprot.org/uniprot/Q99K67

The antiserum was produced against synthesized peptide derived from human AASS. AA range:251-300

AASS Polyclonal Antibody detects endogenous levels of AASS protein.

WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

102kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

Expressed in all 16 tissues examined with highest expression in the liver.

Mitochondrion .

Lysine biosynthesis;Lysine degradation;

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-lysine: step 2/6.,similarity:In the C-terminal section; belongs to the saccharopine dehydrogenase family.,similarity:In the N-terminal section; belongs to the AlaDH/PNT family.,subunit:Homodimer.,tissue specificity:Expressed in all 16 tissues examined with highest expression in the liver.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.