ASL Antilichaam - middengebied: Biotine (ARP41667_T100-Biotine)

Argininosuccinate lyase

ASAL

435

P04424

NP_001020115

Human, Mouse, Rat, Cow, Dog, Goat, Guinea Pig, Horse, Rabbit, Yeast, Zebrafish

The immunogen is a synthetic peptide directed towards the middle region of human ASL

ASL is a member of the lyase 1 family. The protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in its gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency.This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described.

WDYHV1; ASL; GINS4; NCDN; TUFM; PDHA1; NEDD8; HK1; GDI1; BAG3; OVGP1; HMOX1; FBP1; CSNK2A2; UBC; QARS

Biotin

Polyclonal Antibody

WB

0.5 mg/ml

Cow: 100%; Dog: 100%; Goat: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Yeast: 93%; Zebrafish: 93%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

49kDa

Tanaka, T., (2002) Tohoku J. Exp. Med. 198 (2), 119-124

Wet Ice

444

ASL

ASL

Rabbit

Argininosuccinate lyase

NM_001024944

LILYCTKEFSFVQLSDAYSTGSSLMPQKKNPDSLELIRSKAGRVFGREDK