Molecular weight (DA)
150kD
Immunogen
The antiserum was produced against synthesized peptide derived from the Internal region of human PNPLA6. AA range:1031-1080
Specificity
NTE Polyclonal Antibody detects endogenous levels of NTE protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC-p: 1/100-1/300. ELISA: 1/20000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a phospholipase that deacetylates intracellular phosphatidylcholine to produce glycerophosphocholine. It is thought to function in neurite outgrowth and process elongation during neuronal differentiation. The protein is anchored to the cytoplasmic face of the endoplasmic reticulum in both neurons and non-neuronal cells. Mutations in this gene result in autosomal recessive spastic paraplegia, and the protein is the target for neurodegeneration induced by organophosphorus compounds and chemical warfare agents. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009],
Function
catalytic activity:2-lysophosphatidylcholine + H(2)O = glycerophosphocholine + a carboxylate.,disease:Defects in PNPLA6 are the cause of spastic paraplegia autosomal recessive type 39 (SPG39) [MIM:612020]; also known as NTE-related motor neuron disorder (NTEMND). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG39 is associated with a motor axonopathy affecting upper and lower limbs and resulting in progressive wasting of distal upper and
Protein name
Neuropathy target esterase
Other name
PNPLA6; NTE; Neuropathy target esterase; Patatin-like phospholipase domain-containing protein 6
Fields
>>Glycerophospholipid metabolism
Human protein sequence Database
Q8IY17
Mouse protein sequence database
Q3TRM4
Rat protein sequence database
Cellular localization
Endoplasmic reticulum membrane ; Single-pass type III membrane protein .
Tissue expression
Expressed in brain, placenta, kidney, neuron and skeletal muscle. Expressed in the developing eye, pituitary and brain.
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