PRPH
This gene encodes a cytoskeletal protein found in neurons of the peripheral nervous system. The encoded protein is a type III intermediate filament protein with homology to other cytoskeletal proteins such as desmin, and is a different protein that the peripherin found in photoreceptors. Mutations in this gene have been associated with susceptibility to amyotrophic lateral sclerosis. [provided by RefSeq]
Full Name
peripherin
Function
Class-III neuronal intermediate filament protein (By similarity).
May form an independent structural network without the involvement of other neurofilaments or may cooperate with the neuronal intermediate filament proteins NEFL, NEFH, NEFM and INA to form a filamentous network (PubMed:15446584, PubMed:15322088).
Assembly of the neuronal intermediate filaments may be regulated by RAB7A (By similarity).
Plays a role in the development of unmyelinated sensory neurons (By similarity).
May be involved in axon elongation and axon regeneration after injury (By similarity).
Inhibits neurite extension in type II spiral ganglion neurons in the cochlea (By similarity).
May form an independent structural network without the involvement of other neurofilaments or may cooperate with the neuronal intermediate filament proteins NEFL, NEFH, NEFM and INA to form a filamentous network (PubMed:15446584, PubMed:15322088).
Assembly of the neuronal intermediate filaments may be regulated by RAB7A (By similarity).
Plays a role in the development of unmyelinated sensory neurons (By similarity).
May be involved in axon elongation and axon regeneration after injury (By similarity).
Inhibits neurite extension in type II spiral ganglion neurons in the cochlea (By similarity).
Biological Process
Intermediate filament organizationManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Cytoplasm, cytoskeleton
Cell projection, axon
Perikaryon
Cell projection, axon
Perikaryon
Involvement in disease
Amyotrophic lateral sclerosis (ALS):
A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
PTM
Phosphorylated; phosphorylation increases after nerve injury in regenerating neurons.
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Anti-PRPH antibodies
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Target: PRPH
Host: Mouse
Antibody Isotype: IgG
Specificity: Rat, Cattle, Human, Mouse, Pig
Application*: IF, IHC, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: 3B3
Application*: E, P, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG
Specificity: Human, Mouse, Pig, Rat, Cattle
Clone: 2Q135
Application*: IC, IF, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Pig, Rat
Clone: 1H10B3
Application*: E, IH, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1E10
Application*: P
Target: PRPH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Rat
Clone: 13B854
Application*: IC, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat, Mouse, Rabbit, Human
Clone: 8G2
Application*: IF, C, WB
Target: PRPH
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat, Chicken
Clone: 7C5
Application*: IF, P, WB
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For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IGImmunochromatography
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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