AASS Rabbit Polyclonal Antibody

AASS Rabbit Polyclonal Antibody

Size1:50μl Price1:$118
Size2:100μl Price2:$220
Size3:500μl Price3:$980
SKU: APRab06382 Category: Polyclonal Antibody Tags: , ,

Datasheet

Summary

Production Name

AASS Rabbit Polyclonal Antibody

Description

Rabbit Polyclonal Antibody

Host

Rabbit

Application

WB,

Reactivity

Human,Rat,Mouse

 

Performance

Conjugation

Unconjugated

Modification

Unmodified

Isotype

IgG

Clonality

Polyclonal

Form

Liquid

Storage

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Buffer

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

Purification

Affinity purification

 

Immunogen

Gene Name

AASS

Alternative Names

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

Gene ID

10157

SwissProt ID

Q9UDR5

 

Application

Dilution Ratio

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

Molecular Weight

102kD

 

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],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.,

 

Research Area

Lysine biosynthesis;Lysine degradation;