Anti-ASL Antibody (CAB6357)
- SKU:
- CAB6357
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-ASL Antibody |
抗体コード: | CAB6357 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human ASL (NP_000039.2). |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | Mouse liver, Mouse brain, Mouse kidney, Rat liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human ASL (NP_000039.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | MASE SGKL WGGR FVGA VDPI MEKF NASI AYDR HLWE VDVQ GSKA YSRG LEKA GLLT KAEM DQIL HGLD KVAE EWAQ GTFK LNSN DEDI HTAN ERRL KELI GATA GKLH TGRS RNDQ VVTD LRLW MRQT CSTL SGLL WELI RTMV DRAE AERD VLFP GYTH LQRA QPIR WSHW ILSH AVAL TRDS ERLL EVRK RINV LPLG SGAI AGNP LGVD RELL RAEL NFGA ITLN SMDA TSER DFVA EFLF WASL CMTH LSRM AEDL ILYC TKEF SFVQ LSDA YSTG SSLM PQKK NPDS LELI RSKA |
遺伝子ID: | 435 |
Uniprot: | P04424 |
セルラーロケーション: | |
計算された分子量: | 48kDa/49kDa/51kDa |
観察された分子量: | 49KDa |
同義語: | ASL, ASAL |
バックグラウンド: | 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. |
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 |
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