E-AB-90385
ALDH18A1 Polyclonal Antibody
Size
£195.30
Was £217.00
- SKU:
- E-AB-90385
- Additional Names:
- ADCL3,ALDH18A1,ARCL3A,GSAS,P5CS,PYCS,SPG9,SPG9A,SPG9B
- Application:
- WB
- Concentration:
- 1 mg/ml
- Purification:
- Affinity Purified
- Storage Conditions:
- -20[o]C Avoid freeze/thaw cycles.
- Supplier:
- Elabscience
- Host:
- Rabbit
- Reactivities:
- Human, Mouse, Rat
- Buffer:
- Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol.
- Immunogen:
- Recombinant fusion protein of human ALDH18A1
- Uniprot:
- P54886
- Synonyms:
- ADCL3;aldehyde dehydrogenase family 18 member A1;ARCL3A;delta-1-pyrroline-5-carboxylate synthase;delta-1-pyrroline-5-carboxylate synthetase;delta1-pyrroline-5-carboxlate synthetase;GSAS;P5CS;PYCS;pyrroline-5-carboxylate synthetase (glutamate gamma-semialdehyde synthetase);spastic paraplegia 9 (autosomal dominant);Spastic paraplegia-9 (spastic paraparesis with amyotrophy, cataracts and gastroesophageal reflux);SPG9;SPG9A;SPG9B
- Extra Details:
- This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene.
- Shipping Conditions:
- Blue Ice