Product Name	PPT1
Chinese Name	棕榈酰蛋白硫酯酶1单克隆抗体
Alias	CLN1; INCL; Palmitoyl protein hydrolase 1; Palmitoyl protein thioesterase 1; Palmitoyl-protein hydrolase 1; Palmitoyl-protein thioesterase 1; PPT; PPT-1; PPT1; PPT1_HUMAN.
Research Area	Cell biology  Signal transduction
Immunogen Species	Mouse
Clonality	Monoclonal
Clone NO.	10G4
React Species	(predicted: Human, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:20-100 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	31kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PPT1
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008] 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 (PubMed:8816748) 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 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). Similarity: Belongs to the palmitoyl-protein thioesterase family. SWISS: P50897 Gene ID: 5538 Database links: Entrez Gene: 281421 Cow 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: P45479 Rat Unigene: 3873 Human Unigene: 277719 Mouse Unigene: 1574 Rat

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