本公司经销PPT1，棕榈酰蛋白硫酯酶1抗体，克隆类型为polyclonal，宿主来源是Rabbit，PPT1棕榈酰蛋白硫酯酶1抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！
货号：BY-6619R
英文名称：Anti-PPT1
中文名称：棕榈酰蛋白硫酯酶1抗体
其他名称：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.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 34kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat
产品介绍：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.Involvement in disease; Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 （CLN1） . A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, ａnd adult onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by 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）.
