AASS, Polyclonal Antibody

Anti -AASS, ID (Alpha-aminoadipic Semialdehyde Synthase, Mitochondrial, LKR/SDH, Lysine Ketoglutarate Reductase, LKR, LOR, Saccharopine Dehydrogenase)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 7; NC_000007.13 (121713598..121784344, complement). Location: 7q31.3

Polyclonal

IgG

Rabbit

Recognizes human AASS. Species Crossreactivity: mouse

Purified
Purified by ammonium sulfate precipitation

Supplied as a liquid in PBS, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-AASS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

AASS is 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 the gene encoding this protein are associated with familial hyperlysinemia.

Antibodies; Abs to Enzymes, Synthase

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)

Suitable for use in ELISA, Western Blot, and Immunohistochemistry.
Dilution: ELISA: 1:1000
Western Blot: 1:50-1:100
Immunohistochemistry (Paraffn): 1:10-1:50

102,132 Da[Similar Products]

aminoadipate-semialdehyde synthase

alpha-aminoadipic semialdehyde synthase, mitochondrial; OTTHUMP00000211399; OTTHUMP00000211400; OTTHUMP00000211402; lysine-2-oxoglutarate reductase; alpha-aminoadipate semialdehyde synthase; lysine-ketoglutarate reductase /saccharopine dehydrogenase

Alpha-aminoadipic semialdehyde synthase, mitochondrial

AASS_HUMAN

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]

AASS: 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. Defects in AASS are the cause of hyperlysinemia (HYPLYS). Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.

Protein type: Amino Acid Metabolism - lysine degradation; EC 1.5.1.8; Amino Acid Metabolism - lysine biosynthesis; Oxidoreductase; Mitochondrial; EC 1.5.1.9

Chromosomal Location of Human Ortholog: 7q31.3

Cellular Component: mitochondrion; intracellular membrane-bound organelle; mitochondrial matrix

Molecular Function: saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity; saccharopine dehydrogenase (NADP+, L-lysine-forming) activity

Biological Process: L-lysine catabolic process to acetyl-CoA via saccharopine; lysine catabolic process; protein tetramerization

Disease: Saccharopinuria; Hyperlysinemia, Type I

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