Immunohistochemistry - Paraffin (IHC)
(Human Kidney: Formalin-Fixed, Paraffin-Embedded (FFPE))
Rabbit anti-Human PPT1 / CLN1 Polyclonal Antibody | anti-PPT1 / CLN1 antibody
Anti-PPT1 / CLN1 Antibody (aa188-217) IHC-plus
WB (1:1000)
For long term storage store at -20 degree C.
Immunohistochemistry - Paraffin (IHC)
(Human Kidney: Formalin-Fixed, Paraffin-Embedded (FFPE))
Immunohistochemistry - Paraffin (IHC)
(Human Kidney: Formalin-Fixed, Paraffin-Embedded (FFPE))
Western Blot (WB)
(PPT1 Antibody western blot of A549 cell line lysates (35 ug/lane). The PPT1 antibody detected the PPT1 protein (arrow).)
Western Blot (WB)
(PPT1 Antibody western blot of A549 cell line lysates (35 ug/lane). The PPT1 antibody detected the PPT1 protein (arrow).)
Western Blot (WB)
(Western blot of PPT1 (arrow) using rabbit polyclonal PPT1 Antibody. 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the PPT1 gene.)
Western Blot (WB)
(Western blot of PPT1 (arrow) using rabbit polyclonal PPT1 Antibody. 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the PPT1 gene.)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
PPT1: 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. 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). Belongs to the palmitoyl-protein thioesterase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Lipid Metabolism - fatty acid elongation in mitochondria; EC 3.1.2.22; Hydrolase
Chromosomal Location of Human Ortholog: 1p32
Cellular Component: axon; cell soma; cytosol; dendrite; extracellular region; extracellular space; Golgi apparatus; lipid raft; lysosomal lumen; lysosome; membrane; nucleus; synaptic vesicle
Molecular Function: palmitoyl-(protein) hydrolase activity; palmitoyl-CoA hydrolase activity
Biological Process: adult locomotory behavior; associative learning; brain development; cellular lipid metabolic process; cellular protein catabolic process; cofactor metabolic process; cofactor transport; grooming behavior; lipid catabolic process; lipid raft organization and biogenesis; lysosomal lumen acidification; negative regulation of apoptosis; negative regulation of cell growth; negative regulation of neuron apoptosis; nervous system development; neuron development; neurotransmitter secretion; pinocytosis; positive regulation of pinocytosis; positive regulation of receptor-mediated endocytosis; protein catabolic process; protein depalmitoylation; protein transport; receptor-mediated endocytosis; regulation of phospholipase A2 activity; regulation of synapse structure and activity; response to stimulus; sphingolipid catabolic process; triacylglycerol biosynthetic process; visual perception
Disease: Ceroid Lipofuscinosis, Neuronal, 1
Research Articles on PPT1 / CLN1
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Product Notes
The PPT1 / CLN1 ppt1 (Catalog #AAA249659) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-PPT1 / CLN1 Antibody (aa188-217) IHC-plus reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's PPT1 / CLN1 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC) Paraffin, Western Blot (WB). IHC-P (1:100) WB (1:1000). Researchers should empirically determine the suitability of the PPT1 / CLN1 ppt1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PPT1 / CLN1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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