Human Peripheral myelin protein 22 ELISA Kit | PMP22 elisa kit

Human Peripheral myelin protein 22 ELISA Kit

Cat. Num. AAA763805

• Gene Names: PMP22; DSS; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• Reactivity: Human
• Synonyms: Peripheral myelin protein 22; Human Peripheral myelin protein 22 ELISA Kit; PMP22/GAS3/PMP-22/GAS-3/Growth arrest-specific protein 3; PMP22 elisa kit

• Reactivity: Human
• Sequence Length: 160

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 0.156-10ng/ml
• Sensitivity: 0.094ng/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for PMP22 elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human PMP22 antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human PMP22 antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRPStreptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human PMP22 amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Human PMP22 can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 4505907
• NCBI GeneID: 5376
• NCBI Accession #: NP_000295.1
• NCBI GenBank Nucleotide #: NM_000304.3
• 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; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
• NCBI Protein Information: peripheral myelin protein 22
• 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; Cell adhesion; Cell cycle regulation; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 17p12

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

Molecular Function: protein binding

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

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