琥珀酸裂解酶抗体
规格：1尘驳/1尘濒
英文名： Argininosuccinate Lyase
别名： Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1.
分子量： 52kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ASL/Argi
交叉反应：Human, Mouse, Rat, Dog, Pig, Horse,
细胞定位：细胞外基质
琥珀酸裂解酶抗体产物介绍：background: 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] Function: Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 3/3. Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)琥珀酸裂解酶抗体succinate: step 1/1. Subcellular Location: Acetylation modifies enzyme activity in response to alterations of extracellular nutrient availability. Acetylation increased with trichostin A (TSA) or with nicotinamide (NAM). Glucose increases acetylation by about a factor of 3 with decreasing enzyme activity. Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity. DISEASE: Defects in ASL are the cause of arginosuccinicaciduria (ARGINSA) [MIM:207900]. Arginosuccinicaciduria is an autosomal recessive disorder琥珀酸裂解酶抗体 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. Similarity: Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. Database links: Entrez Gene: 435 Human Entrez Gene: 109900 Mouse Entrez Gene: 59085 Rat Entrez Gene: 512771 Cow Omim: 608310 Human SwissProt: Q3SZJ0 Cow SwissProt: P04424 Human SwissProt: Q91YI0 Mouse SwissProt: P20673 Rat Unigene: 632015 Human Unigene: 23869 Mouse Unigene: 64591 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
琥珀酸裂解酶抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid