含patatin样磷脂酶6抗体

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英文名称  Anti-PNPLA6/NTE

中文名称  含patatin样磷脂酶6抗体

别    名  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.

浓    度  1mg/1ml

规 格  0.1ml/100μg  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse

产品类型  一抗    

研究领域  细胞生物 神经生物学 信号转导 激酶和磷酸酶  

蛋白分子量  predicted molecular weight: 150kDa

性    状  Lyophilized or Liquid

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免 疫 原  KLH conjugated synthetic peptide derived from human PNPLA6

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500

（石蜡切片需做抗原修复）

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品介绍 Phospholipase B that deacylates intracellular phosphatidylcholine (PtdCho), generating glycerophosphocholine (GroPtdCho). This deacylation occurs at both sn-2 and sn-1 positions of PtdCho. 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 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 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 lower extremity muscles.

Function : Phospholipase B that deacylates intracellular phosphatidylcholine (PtdCho), generating glycerophosphocholine (GroPtdCho). This deacylation occurs at both sn-2 and sn-1 organophosphorus (OP) compounds leads to distal axonopathy.

Subcellular Location : Endoplasmic reticulum membrane; Single-pass ype I membrane protein; Cytoplasmic side. Note=Anchored to the cytoplasmic face of the endoplasmic reticulum by its amino-terminal transmembrane segment.

Tissue Specificity : Expressed in brain, placenta, kidney, neuron and skeletal muscle.

Post-translational modifications : Glycosylated.

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 lower extremity muscles.

含patatin样磷脂酶6抗体Similarity : Belongs to the NTE family.

Contains 3 cyclic nucleotide-binding domains.

Contains 1 patatin domain.