Key features and details
- Rabbit polyclonal to PMP22
- Suitable for: IHC-P
- Reacts with: Rat
- Isotype: IgG
Product nameAnti-PMP22 antibody
See all PMP22 primary antibodies
DescriptionRabbit polyclonal to PMP22
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Rat
Predicted to work with: Mouse, Human
- IHC-P: Rat brain and spinal cord tissues.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 50% Glycerol, 1% BSA
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab203053 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionMight be involved in growth regulation, and in myelinization in the peripheral nervous system.
Involvement in diseaseDefects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A) [MIM:118220]; also known as hereditary motor and sensory neuropathy IA. CMT1A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. CMT1A inheritance is autosomal dominant.
Defects in PMP22 are a cause of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.
Defects in PMP22 are a cause of hereditary neuropathy with liability to pressure palsies (HNPP) [MIM:162500]; an autosomal dominant disorder characterized by transient episodes of decreased perception or peripheral nerve palsies after slight traction, compression or minor traumas.
Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1E (CMT1E) [MIM:118300]; also known as Charcot-Marie-Tooth disease and deafness autosomal dominant. CMT1E is an autosomal dominant form of Charcot-Marie-Tooth disease characterized by the association of sensorineural hearing loss with peripheral demyelinating neuropathy.
Defects in PMP22 may be a cause of inflammatory demyelinating polyneuropathy (IDP) [MIM:139393]. IDP is a putative autoimmune disorder presenting in an acute (AIDP) or chronic form (CIDP). The acute form is also known as Guillain-Barre syndrome.
Sequence similaritiesBelongs to the PMP-22/EMP/MP20 family.
- Information by UniProt
- CMT1A antibody
- CMT1E antibody
- DSS antibody
Immunohistochemical analysis of formalin-fixed paraffin-embedded rat brain tissue, labeling PMP22 using ab203053 at a 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.
Immunohistochemical analysis of formalin-fixed paraffin-embedded rat spinal cord tissue, labeling PMP22 using ab203053 at a 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.
ab203053 has not yet been referenced specifically in any publications.