ASL Antibody, HRP conjugated (PACO25093)
- SKU:
- PACO25093
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | ASL Antibody, HRP conjugated (PACO25093) |
抗体コード: | PACO25093 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Argininosuccinate lyase protein (2-300AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | HRP |
バックグラウンド: | cytoplasm, cytosol, extracellular exosome, argininosuccinate lyase activity, arginine biosynthetic process, arginine catabolic process, cellular nitrogen compound metabolic process, internal protein amino acid, acetylation, polyamine metabolic process, protein tetramerization |
シノニム: | Argininosuccinate lyase (ASAL) (EC 4.3.2.1) (Arginosuccinase), ASL |
UniProt Protein Function: | 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. |
UniProt Protein Details: | 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 |
NCBI Summary: | 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 Code: | P04424 |
NCBI GenInfo Identifier: | 124028641 |
NCBI Gene ID: | 435 |
NCBI Accession: | P04424.4 |
UniProt Secondary Accession: | P04424,Q6LDS5, Q96HS2, E7EMI0, E9PE48, |
UniProt Related Accession: | P04424 |
Molecular Weight: | 464 |
NCBI Full Name: | Argininosuccinate lyase |
NCBI Synonym Full Names: | argininosuccinate lyase |
NCBI Official Symbol: | ASL |
NCBI Official Synonym Symbols: | ASAL |
NCBI Protein Information: | argininosuccinate lyase; arginosuccinase; argininosuccinase |
UniProt Protein Name: | Argininosuccinate lyase |
UniProt Synonym Protein Names: | Arginosuccinase |
UniProt Gene Name: | ASL |
UniProt Entry Name: | ARLY_HUMAN |