棕榈酰蛋白硫酯酶1抗体
规格：1尘驳/1尘濒
英文名： PPT1
别名： CLN1; INCL; Palmitoyl protein hydrolase 1; Palmitoyl protein thioesterase 1; Palmitoyl-protein hydrolase 1; Palmitoyl-protein thioesterase 1; PPT; PPT-1; PPT1; PPT1_HUMAN.
分子量： 31kDa
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 PPT1 (20
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
棕榈酰蛋白硫酯酶1抗体产物介绍：background: Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons. Function: Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons. Subcellular Location: Lysosome. DISEASE: Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1) [MIM:256730]. A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, and onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by棕榈酰蛋白硫酯酶1抗体 intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). Similarity: Belongs to the palmitoyl-protein thioesterase family. Database links: Entrez Gene: 281421 Cow Entrez Gene: 475316 Dog Entrez Gene: 5538 Human Entrez Gene: 19063 Mouse Entrez Gene: 29411 Rat Omim: 600722 Human SwissProt: P45478 Cow SwissProt: P50897 Human SwissProt: O88531 Mouse SwissProt: 棕榈酰蛋白硫酯酶1抗体P45479 Rat Unigene: 3873 Human Unigene: 277719 Mouse Unigene: 1574 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
棕榈酰蛋白硫酯酶1抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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：细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid