ITT0975
ITT0975
- Catalog: ITT0975
- Gene/Protein: PPT1
- Product Description: Immunotag™ CLN1 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | CLN1 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | IHC-p,ELISA |
| Recommended Dilution | Immunohistochemistry: 1/100 - 1/300. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from the N-terminal region of human CLN1 |
| Specificity | CLN1 Polyclonal Antibody detects endogenous levels of CLN1 protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | PPT1 |
| Accession No. | P50897 O88531 P45479 |
| Alternate Names | PPT1; PPT; Palmitoyl-protein thioesterase 1; PPT-1; Palmitoyl-protein hydrolase 1 |
| Description | palmitoyl-protein thioesterase 1(PPT1) Homo sapiens 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], |
| Cell Pathway/ Category | Fatty acid elongation in mitochondria,Lysosome, |
| Protein Expression | Brain,Cerebellum,Liver,Prostate,Testis, |
| Subcellular Localization | extracellular region,extracellular space,nucleus,lysosome,Golgi apparatus,cytosol,synaptic vesicle,membrane,axon,dendrite,neuronal cell body,lysosomal lumen,membrane raft,extracellular exosome, |
| Protein Function | catalytic activity:Palmitoyl-protein + H(2)O = palmitate + protein.,disease:Defects in PPT1 are a cause of neuronal ceroid lipofuscinosis 4 (CLN4) [MIM:204300]; also known as adult type neuronal ceroid lipofuscinosis (NCL) or Kufs disease.,disease:Defects in PPT1 are the cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1) [MIM:256730]; also called infantile neuronal ceroid lipofuscinosis (INCL). The neuronal ceroid lipofuscinosis are a group of progressive neurodegenerative diseases characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). There is a core group of four major clinical forms, the infantile, the late-infantile, the juvenile, and the adult forms. The infantile forms are characterized by progressive visual impairment, seizure, motor disturbances, dementia and premature death (8-11 years of age).,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.,online information:Neural Ceroid Lipofuscinoses mutation db,online information:Retina International's Scientific Newsletter,similarity:Belongs to the palmitoyl-protein thioesterase family., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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