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ASL Antibody - N-terminal region : FITC (ARP41666_P050-FITC)

Product Specifications

Gene Name

Argininosuccinate lyase

Gene Aliases

ASAL

Gene ID

435

Swiss Prot

P04424

Accession Number

NP_000039

Host

Rabbit

Reactivity

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

Immunogen

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

Target

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.

Partner Proteins

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

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

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

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

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.

Molecular Weight

52kDa

References & Citations

Trevisson, E., (2007) Hum. Mutat. 28 (7), 694-702

Shipping Conditions

Wet Ice

Protein Length

464

NCBI Gene Symbol

ASL

NCBI GB Accession Number

ASL

Host or Source

Rabbit

Protein Name

Argininosuccinate lyase

Nucleotide Accession Number

NM_000048

Peptide Sequence

GATAGKLHTGRSRNDQVVTDLRLWMRQTCSTLSGLLWELIRTMVDRAEAE

Frequently Asked Questions

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