Rabbit anti-Human, Mouse ASL Polyclonal Antibody | anti-ASL antibody

ASL (Arginosuccinase, Argininosuccinate Lyase, ASAL) (PE)

Cat. Num. AAA6370506

• Gene Names: ASL; ASAL
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ASL; Polyclonal Antibody; ASL (Arginosuccinase; Argininosuccinate Lyase; ASAL) (PE); anti-ASL antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ASL. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-ASL antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human ASL, aa1-464 (NP_000039.2).
• Immunogen Sequence: MASESGKLWGGRFVGAVDPIMEKFNASIAYDRHLWEVDVQGSKAYSRGLEKAGLLTKAEMDQILHGLDKVAEEWAQGTFKLNSNDEDIHTANERRLKELIGATAGKLHTGRSRNDQVVTDLRLWMRQTCSTLSGLLWELIRTMVDRAEAERDVLFPGYTHLQRAQPIRWSHWILSHAVALTRDSERLLEVRKRINVLPLGSGAIAGNPLGVDRELLRAELNFGAITLNSMDATSERDFVAEFLFWASLCMTHLSRMAEDLILYCTKEFSFVQLSDAYSTGSSLMPQKKNPDSLELIRSKAGRVFGRCAGLLMTLKGLPSTYNKDLQEDKEAVFEVSDTMSAVLQVATGVISTLQIHQENMGQALSPDMLATDLAYYLVRKGMPFRQAHEASGKAVFMAETKGVALNQLSLQELQTISPLFSGDVICVWDYGHSVEQYGALGGTARSSVDWQIRQVRALLQAQQA
• Conjugate: PE
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(ASL rabbit polyclonal antibody. Western Blot analysis of ASL expression in human liver.)

Western Blot (WB)

(ASL rabbit polyclonal antibody. Western Blot analysis of ASL expression in mouse kidney.)

Western Blot (WB)

(Western Blot analysis of ASL expression in transfected 293T cell line by ASL polyclonal antibody. Lane 1: ASL transfected lysate (51.7kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-ASL antibody

ASL is a member of the lyase 1 family. This 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.

Product Categories/Family for anti-ASL antibody

Antibodies; Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 31541964
• NCBI GeneID: 435
• NCBI Accession #: NP_000039.2
• NCBI GenBank Nucleotide #: NM_000048.3
• UniProt Accession #: P04424; Q6LDS5; Q96HS2; E7EMI0; E9PE48
• Molecular Weight: 48,733 Da
• NCBI Official Full Name: argininosuccinate lyase isoform 1
• NCBI Official Synonym Full Names: argininosuccinate lyase
• NCBI Official Symbol: ASL
• NCBI Official Synonym Symbols: ASAL
• NCBI Protein Information: argininosuccinate lyase; argininosuccinase; arginosuccinase
• UniProt Protein Name: Argininosuccinate lyase
• UniProt Gene Name: ASL
• UniProt Synonym Gene Names: ASAL
• UniProt Entry Name: ARLY_HUMAN

NCBI Description

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. [provided by RefSeq, Jul 2008]

Uniprot Description

ASL: Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA). An autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness. Belongs to the lyase 1 family. Argininosuccinate lyase subfamily.

Protein type: Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 4.3.2.1; Amino Acid Metabolism - arginine and proline

Chromosomal Location of Human Ortholog: 7q11.21

Cellular Component: cytoplasm; cytosol

Molecular Function: argininosuccinate lyase activity

Biological Process: arginine catabolic process; arginine biosynthetic process via ornithine; internal protein amino acid acetylation; urea cycle

Disease: Argininosuccinic Aciduria