Rabbit anti-Human, Rat PMP22 Monoclonal Antibody | anti-PMP22 antibody

PMP22 Antibody

Cat. Num. AAA9436351

• Gene Names: PMP22; DSS; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• Reactivity: Human, Rat
• Applications: Western Blot, Immunocytochemistry, Immunohistochemistry, Flow Cytometry, Functional Assay
• Purity: ProA affinity purified
• Synonyms: PMP22; Monoclonal Antibody; PMP22 Antibody; CMT1A antibody; CMT1E antibody; DSS antibody; GAS-3 antibody; GAS3 antibody; Growth Arrest Specific 3 antibody; Growth arrest-specific protein 3 antibody; HMSNIA antibody; HNPP antibody; MGC20769 antibody; Peripheral myelin protein 22 antibody; PMP-22 antibody; PMP22 antibody; PMP22_HUMAN antibody; Sp110 antibody; Trembler antibody; anti-PMP22 antibody

• Host: Rabbit
• Reactivity: Human, Rat
• Clonality: Monoclonal
• Clone Number: JM52-30
• Purity/Purification: ProA affinity purified
• Form/Format: 1*TBS (pH7.4), 1% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

• Applicable Applications for anti-PMP22 antibody: Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)
• Application Notes: WB: 1:500; IHC: 1:50-1:200; ICC: 1:50-1:200; FC/FACS: 1:50-1:100
• Antibody Type: Recombinant Antibody
• Immunogen: Recombinant protein
• Conjugation: Unconjugated
• Preparation and Storage: Store at -20 degree C.

Western Blot (WB)

(Western blot analysis of PMP22 on PC-12 cell using anti-PMP22 antibody at 1/500 dilution.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded rat brain tissue using anti-PMP22 antibody. Counter stained with hematoxylin.)

Immunocytochemistry (ICC)

(ICC staining PMP22 in N2A cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of SH-SY5Y cells with PMP22 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black).)

Related Product Information for anti-PMP22 antibody

PLP (myelin proteolipid protein or lipophilin) is a major constituent of myelin. The two isoforms of the myelin proteolipid protein, PLP and DM20, are very hydrophobic integral membrane proteins that account for about half of the protein content of adult CNS myelin. A mutation in the gene which encodes PLP is linked to Pelizaeus-Merzbacher disease (PMD), a chronic infantile type of diffuse cerebral sclerosis. The gene which encodes PLP maps to human chromosome Xq13-q22. The glycoprotein zero (also designated P-zero or myelin peripheral protein) is the major structural protein of peripheral myelin, accounting for more than 50% of the protein present in the sheath of peri-pheral nerves. Zero is an integral membrane glycoprotein whose expression is restricted to Schwann cells. The gene which encodes zero maps to human chromosome 1q22. PMP22 (peripheral myelin protein 22) is a growth-regulated membrane protein which is expressed by Schwann cells and is localized mainly in compact peripheral nervous system myelin. The gene which encodes PMP22 maps to human chromosome 17p11.2.

Product Categories/Family for anti-PMP22 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4505907
• NCBI GeneID: 5376
• NCBI Accession #: NP_000295.1
• NCBI GenBank Nucleotide #: NM_000304.3
• UniProt Accession #: Q01453; Q8WV01
• Molecular Weight: 17,891 Da
• NCBI Official Full Name: peripheral myelin protein 22
• NCBI Official Synonym Full Names: peripheral myelin protein 22
• NCBI Official Symbol: PMP22
• NCBI Official Synonym Symbols: DSS; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• NCBI Protein Information: peripheral myelin protein 22; growth arrest-specific protein 3
• UniProt Protein Name: Peripheral myelin protein 22
• Protein Family: Peripheral myelin protein
• UniProt Gene Name: PMP22
• UniProt Synonym Gene Names: GAS3; PMP-22; GAS-3
• UniProt Entry Name: PMP22_HUMAN

NCBI Description

This gene encodes an integral membrane protein that is a major component of myelin in the peripheral nervous system. Studies suggest two alternately used promoters drive tissue-specific expression. Various mutations of this gene are causes of Charcot-Marie-Tooth disease Type IA, Dejerine-Sottas syndrome, and hereditary neuropathy with liability to pressure palsies. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

Uniprot Description

PMP22: Might be involved in growth regulation, and in myelinization in the peripheral nervous system. Defects in PMP22 are the cause of Charcot-Marie-Tooth disease type 1A (CMT1A); 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); 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); 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); 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). 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. Belongs to the PMP-22/EMP/MP20 family.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Cell cycle regulation; Cell adhesion

Chromosomal Location of Human Ortholog: 17p12

Cellular Component: compact myelin; tight junction; integral to membrane; plasma membrane

Molecular Function: protein binding

Biological Process: cell death; negative regulation of cell proliferation; myelin formation; synaptic transmission; bleb formation; peripheral nervous system development

Disease: Charcot-marie-tooth Disease, Demyelinating, Type 1a; Neuropathy, Hereditary, With Liability To Pressure Palsies; Hypertrophic Neuropathy Of Dejerine-sottas; Charcot-marie-tooth Disease And Deafness; Roussy-levy Hereditary Areflexic Dystasia; Guillain-barre Syndrome, Familial