Anti-PNPLA6/NTE/FITC Conjugated抗体上海钰博生物科技有限公司

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产品名称: Anti-PNPLA6/NTE/FITC Conjugated抗体
英文名称: Anti-PNPLA6/NTE/FITC
抗体货号: YB--6723R-FITC
产品规格: 20ul/50ul/100ul/200ul
级    别: , 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 2980元
抗原: KLH conjugated synthetic peptide derived from human PNPLA6
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/4/17 9:38:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-PNPLA6/NTE/FITC Conjugated抗体

产品编号 YB-6723R-FITC
英文名称 Anti-PNPLA6/NTE/FITC
中文名称 FITC标记的含patatin样***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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  激酶和***酸酶  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 150kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
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 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
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.

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.

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

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