含patatin样磷脂酶6抗体
规格：1mg/1ml
英文名： PNPLA6/NTE
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; PLPL
分子量： 150kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PNPLA6
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
细胞定位：细胞浆 细胞膜
含patatin样磷脂酶6抗体产品介绍：background: 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. 含patatin样磷脂酶6抗体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,含patatin样磷脂酶6抗体 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. Similarity: Belongs to the NTE family. Contains 3 cyclic nucleotide-binding domains. Contains 1 patatin domain. Database links: Entrez Gene: 10908 Human Entrez Gene: 50767 Mouse Omim: 603197 Human SwissProt: Q8IY17 Human SwissProt: Q3TRM4 Mouse Unigene: 631863 Human Unigene: 23085 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
含patatin样磷脂酶6抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：细胞生物 神经生物学 信号转导 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid