2. Gene
  3. Aass - aminoadipate-semialdehyde synthase Gene

Aass - aminoadipate-semialdehyde synthase Gene

  • Gene
  • Protein
  • Agent
  • Ortholog
Mus musculus

Also known as LKR; LOR; SDH; Lorsdh; LKR/SDH; LOR/SDH

Gene ID: 30956 | Gene type: protein coding

About Aass

Summary

This gene encodes a bifunctional mitochondrial protein that catalyzes the first two steps in the lysine degradation pathway. The N-terminus contains lysine-ketoglutarate reductase activity and converts lysine to saccharopine, whereas the C-terminus contains saccharopine dehydrogenase activity and converts saccharopine to alpha-aminoadipate semialdehyde. Mutations in a human gene encoding a highly similar protein are associated with familial hyperlysinemia. [provided by RefSeq, Jul 2008]

Aass Products(1)

mRNA Protein Name
NM_013930.4 NP_038958.2 alpha-aminoadipic semialdehyde synthase, mitochondrial
Gene Ontology
  • Molecular Function
  • Biological Process
Molecular Function GO Annotation Evidence Reference Source
enables saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity IDA
IDA: Inferred from direct assay
10567240 MGI
enables saccharopine dehydrogenase (NADP+, L-lysine-forming) activity IDA
IDA: Inferred from direct assay
10567240 MGI
Biological Process GO Annotation Evidence Reference Source
acts upstream of or within L-lysine catabolic process IDA
IDA: Inferred from direct assay
10567240 MGI
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern
Protein Preferred Names Protein Names

alpha-aminoadipic semialdehyde synthase, mitochondrial

lysine oxoglutarate reductase

saccharopine dehydrogenase

Related Agents

Pre-clinical Phase
Bioactive Molecules (2)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-159681 AASS-IN-1 98.40% Unkown
HY-RS00033 AASS Human Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Homo sapiens Aass NCBI NCBI:10157