Human ASL Recombinant Protein (RPPB1417)
- SKU:
- RPPB1417
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P04424
- Research Area:
- Enzymes
Description
| Product Name: | Human ASL Recombinant Protein |
| Product Code: | RPPB1417 |
| Size: | 20µg |
| Species: | Human |
| Target: | ASL |
| Synonyms: | Argininosuccinate lyase, ASAL, Arginosuccinase, ASL. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The ASL solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 2mM DTT, 10% glycerol and 100mM NaCl. |
| Stability: | NPL Human Recombinant although stable at 4°C for 1 week, should be stored below -18°C. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Please prevent freeze thaw cycles. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MASESGKLWG GRFVGAVDPI MEKFNASIAY DRHLWEVDVQ GSKAYSRGLE KAGLLTKAEM DQILHGLDKV AEEWAQGTFK LNSNDEDIHT ANERRLKELI GATAGKLHTG RSRNDQVVTD LRLWMRQTCS TLSGLLWELI RTMVDRAEAE RDVLFPGYTH LQRAQPIRWS HWILSHAVAL TRDSERLLEV RKRINVLPLG SGAIAGNPLG VDRELLRAEL NFGAITLNSM DATSERDFVA EFLFWASLCM THLSRMAEDL ILYCTKEFSF VQLSDAYSTG SSLMPQKKNP DSLELIRSKA GRVFGRCAGL LMTLKGLPST YNKDLQEDKE AVFEVSDTMS AVLQVATGVI STLQIHQENM GQALSPDMLA TDLAYYLVRK GMPFRQAHEA SGKAVFMAET KGVALNQLSL QELQTISPLF SGDVICVWDY GHSVEQYGAL GGTARSSVDW QIRQVRALLQ AQQA |
Argininosuccinate lyase (ASL) is a member of the lyase 1 family. ASL is an enzyme which catalyzes the reversible breakdown of Argininosuccinate (ASA) yielding the amino acids arginine and fumarate. ASL which is located in the liver cytosol is the 4th enzyme of the urea cycle and involved in the biosynthesis of arginine in all species and the production of urea in ureotelic species. While Argininosuccinate synthetase (ASS) catalyzes the formation of argininosuccinate from citrulline and aspartate, ASL breaks down the newly formed argininosuccinate into L-arginine and fumarate. L-arginine continues within the urea cycle to form urea and orinthine, whereas fumarate can enter the citric acid cycle. ASL gene Mutations result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency.
ASL Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 484 amino acids (1-464) and having a molecular mass of 53.8kDa.ASL is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| 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 |
