含patatin样磷脂酶6抗体

Quantity size:

0.2ml

Concentration:

1mg/ml含patatin样磷脂酶6抗体   Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Phospholipase B that deacylates intracellular phosphatidylcholine (PtdCho), generating glycerophosphocholine (GroPtdCho). This deacylation occurs at both sn-2 and sn-1 positions of PtdCho.含patatin样磷脂酶6抗体 Its specific chemical modification by certain organophosphorus (OP) compounds leads to distal axonopathy.
Tissue specificity;Expressed in brain, placenta, kidney, neuron and skeletal muscle.
Involvement in diseaseDefects in PNPLA6 are the cause of spastic paraplegia 含patatin样磷脂酶6抗体autosomal recessive type 39 (SPG39) ; 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 t含patatin样磷脂酶6抗体o 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 lower extremity muscles.

Also known as:

Neuropathy target esterase; NTEMND; Patatin like phospholipase domain containing 6; Patatin like phospholipase domain containing protein 6; Patatin-like phospholipase domain-containing protein 6; PLPL6_HUMAN; Pnpla6; SPG39; sws.

含patatin样磷脂酶6抗体Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, .

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Immunogen: KLH conjugated synthetic peptide derived from human PNPLA6.

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Predicted Molecular Weight: 150kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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