Application
Western blot analysis of CLN1 expression in 293 whole cell lysate.Application
Immunoperoxidase staining of formalin-fixed, paraffin-embedded human hepatocarcinoma tissue showing cytoplasmic localization.| Product Name | CLN1 Antibody (O-21) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | CLN1 (ceroid lipofuscinosis 1), also known as PPT, INCL or PPT1 (palmitoyl-protein thioesterase 1), is a 306 amino acid glycosylated protein that localizes to lysosome and is a member of the palmitoyl-protein thioesterase family. CLN1 functions to remove thioester-linked fatty acyl groups from a variety of substrates, such as as palmitate, from modified cysteine residues in proteins or peptides during lysosomal degradation. Defects in the gene encoding CLN1 are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1 or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, with clinical symptoms including seizures, dementia, visual loss and/or cerebral atrophy. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFIHCELISA |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | recommended for detection of CLN1 of human origin by WB IP IF IHC(P) and ELISA |
|---|---|
| Tissue Specificity | recommended for detection of CLN1 of human origin by WB, IP, IF, IHC(P) and ELISA |
| Entrez Gene | 5538 |
|---|
Application
Western blot analysis of CLN1 expression in 293 whole cell lysate.
Application
Immunoperoxidase staining of formalin-fixed, paraffin-embedded human hepatocarcinoma tissue showing cytoplasmic localization.| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
