SLC19A3 antibody, Internal-多抗-深圳欣博盛生物科技有限公司

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SLC19A3 antibody, Internal

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品牌：GeneTex

官网链接

说明书

折扣/货期

货号：GTX46980

规格：50μg

目录价：￥6000

产品详情

* 以下信息仅供参考，详情请以原厂网站为准

产品名称:

SLC19A3 antibody, Internal

别名:

solute carrier family 19 member 3 , BBGD , THMD2 , THTR2

反应种属:

Human

宿主来源:

Rabbit

实验应用:

同种型:

IgG

免疫原:

A synthetic peptide corresponding to an Internal region of Human SLC19A3

克隆性:

Polyclonal

克隆号:

纯化方式:

Affinity Purified

偶联:

Unconjugated

产品浓度:

0.5-1 mg/ml (Please refer to the vial label for the specific concentration.)

保存温度:

Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.

运输温度:

4°C

预期分子量:

产品形式:

Liquid

存储溶液:

PBS, 2% Sucrose, 0.09% Sodium azide.

生产商:

GeneTex

功能与背景:

This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild cognitive disability, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy.[provided by RefSeq, Jan 2010]

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