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E-AB-90385

ALDH18A1 Polyclonal Antibody

Größe

£195.30

War £217.00

SKU:
E-AB-90385
Zusätzliche Namen:
ADCL3,ALDH18A1,ARCL3A,GSAS,P5CS,PYCS,SPG9,SPG9A,SPG9B
Anwendung:
WB
Konzentration:
1 mg/ml
Aufreinigung:
Affinity Purified
Lagerbedingungen:
-20[o]C Avoid freeze/thaw cycles.
Hersteller:
Elabscience
Host:
Rabbit
Reaktivitäten:
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
Synonyme:
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
Weitere 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.
Versandbedingungen:
Blue Ice