Rabbit anti-Human, Mouse AASS Polyclonal Antibody | anti-AASS antibody

AASS, ID (Alpha-aminoadipic Semialdehyde Synthase, Mitochondrial, LKR/SDH, Lysine Ketoglutarate Reductase, LKR, LOR, Saccharopine Dehydrogenase) (MaxLight 750)

Cat. Num. AAA6460813

• Gene Names: AASS; LKRSDH; LORSDH; LKR/SDH
• Reactivity: Human, Mouse
• Applications: Western Blot, Immunohistochemistry
• Purity: Purified by Ammonium Sulfate Precipitation
• Synonyms: AASS; Polyclonal Antibody; ID (Alpha-aminoadipic Semialdehyde Synthase; Mitochondrial; LKR/SDH; Lysine Ketoglutarate Reductase; LKR; LOR; Saccharopine Dehydrogenase) (MaxLight 750); anti-AASS antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human AASS. Species Crossreactivity: mouse
• Purity/Purification: Purified by Ammonium Sulfate Precipitation
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight750.
• Sequence Length: 926

• Applicable Applications for anti-AASS antibody: Western Blot (WB), FLISA, Immunohistochemistry (IHC)
• Application Notes: WB: 1:50-1:100; FLISA: 1:1000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide corresponding to a portion of amino acids within aa272-301 from the center region of human AASS. conjugated to KLH.
• Conjugate: MaxLight750
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight750 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of AASS Antibody (Center) in 293 cell line lysates (35ug/lane). AASS (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with the AASS antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Western Blot (WB)

(AASS Antibody (Center) western blot analysis in mouse liver tissue lysates (35ug/lane).This demonstrates the AASS antibody detected the AASS protein (arrow).)

Product Categories/Family for anti-AASS antibody

Antibodies; Enzymes; Synthase

References

1. Sacksteder, K.A., Am. J. Hum. Genet. 66(6), 1736-1743 (2000).

NCBI and Uniprot Product Information

• NCBI GI #: 13027640
• NCBI GeneID: 10157
• NCBI Accession #: NP_005754
• NCBI GenBank Nucleotide #: NP_005754.2
• UniProt Accession #: Q9UDR5
• Molecular Weight: 102.001
• NCBI Official Full Name: alpha-aminoadipic semialdehyde synthase, mitochondrial
• NCBI Official Synonym Full Names: aminoadipate-semialdehyde synthase
• NCBI Official Symbol: AASS
• NCBI Official Synonym Symbols: LKRSDH; LORSDH; LKR/SDH
• NCBI Protein Information: alpha-aminoadipic semialdehyde synthase, mitochondrial
• UniProt Protein Name: Alpha-aminoadipic semialdehyde synthase, mitochondrial
• UniProt Gene Name: AASS
• UniProt Synonym Gene Names: LKR; LOR; SDH
• UniProt Entry Name: AASS_HUMAN

NCBI Description

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]

Uniprot Description

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

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