Sign in or Register   Sign in or Register
  |  

PPT1

PPT1 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).
Full Name
palmitoyl-protein thioesterase 1
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).
Biological Process
Adult locomotory behaviorIEA:Ensembl
Associative learningIEA:Ensembl
Brain developmentManual Assertion Based On ExperimentIMP:UniProtKB
Cellular macromolecule catabolic processIEA:Ensembl
Fatty-acyl-CoA biosynthetic processTAS:Reactome
Grooming behaviorIEA:Ensembl
Lipid catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Lysosomal lumen acidificationManual Assertion Based On ExperimentIMP:UniProtKB
Membrane raft organizationManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of apoptotic processManual Assertion Based On ExperimentIDA:UniProtKB
Negative regulation of cell growthManual Assertion Based On ExperimentIMP:UniProtKB
Negative regulation of neuron apoptotic processManual Assertion Based On ExperimentIMP:UniProtKB
Nervous system developmentManual Assertion Based On ExperimentIMP:UniProtKB
Neuron developmentManual Assertion Based On ExperimentTAS:UniProtKB
Neurotransmitter secretionIEA:Ensembl
PinocytosisManual Assertion Based On ExperimentIMP:MGI
Positive regulation of pinocytosisManual Assertion Based On ExperimentIMP:UniProtKB
Positive regulation of receptor-mediated endocytosisManual Assertion Based On ExperimentIMP:UniProtKB
Protein catabolic process1 PublicationNAS:UniProtKB
Protein depalmitoylationManual Assertion Based On ExperimentIDA:UniProtKB
Protein transportManual Assertion Based On ExperimentIMP:UniProtKB
Receptor-mediated endocytosisManual Assertion Based On ExperimentIMP:MGI
Regulation of phospholipase A2 activityIEA:Ensembl
Regulation of synapse structure or activity1 PublicationNAS:UniProtKB
Response to stimulusIEA:UniProtKB-KW
Sphingolipid catabolic processManual Assertion Based On ExperimentTAS:UniProtKB
Visual perceptionIEA:UniProtKB-KW
Cellular Location
Lysosome
Secreted
Involvement in disease
Ceroid lipofuscinosis, neuronal, 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).
PTM
Glycosylated.

Anti-PPT1 antibodies

Loading...
Target: PPT1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 10F3
Application*: FC, IHC-P, WB
Target: PPT1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1F10
Application*: WB, IH, P
Target: PPT1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 1A10
Application*: P, WB
Target: PPT1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1117CT11.2.1.4
Application*: WB, P
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
Go to
Compare