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顶部产品介绍抗原信息化学信息储存和溶解信息计算器

Anti-PANK2 Polyclonal Antibody

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货号 TMAB-09962

Anti-PANK2 Polyclonal Antibody 是一种 Rabbit 抗体，靶向 PANK2。Anti-PANK2 Polyclonal Antibody 可用于 WB。

Anti-PANK2 Polyclonal Antibody

Anti-PANK2 Polyclonal Antibody

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Rating icon 还可以
货号 TMAB-09962

Anti-PANK2 Polyclonal Antibody 是一种 Rabbit 抗体，靶向 PANK2。Anti-PANK2 Polyclonal Antibody 可用于 WB。

规格价格库存数量
50 μL
¥ 1,170
5日内发货
100 μL
¥ 1,965
5日内发货
200 μL
¥ 2,795
5日内发货
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产品介绍

生物活性
产品描述
Anti-PANK2 Polyclonal Antibody is a Rabbit antibody targeting PANK2. Anti-PANK2 Polyclonal Antibody can be used in WB.
Ig Type
IgG
反应种属
Human,Mouse (predicted:Rat,Dog,Pig,Cow,Horse)
应用WB
推荐剂量
WB: 1:500-2000
抗体种类
Polyclonal
宿主来源Rabbit
亚细胞定位Isoform 1: Mitochondrion. Isoform 2: Cytoplasm (Potential).
组织特异性Ubiquitous.
构建方式Hybridoma Polyclonal Antibody
纯化方式Protein A purified
性状Liquid
缓冲液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
浓度1mg/ml
研究背景Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI. Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
抗原信息
免疫原
KLH conjugated synthetic peptide: human PANK2
抗原种属
Human
基因名称
PANK2
基因ID
80025
蛋白名称
Pantothenate kinase 2, mitochondrial
Uniprot ID
Q9BZ23
功能
May be the master regulator of the CoA biosynthesis (By similarity).
化学信息
分子量Theoretical: 57 kDa.
储存&溶解度
储存方式Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles.
运输方式Shipping with blue ice.
存储store at low temperature | -20°C for 1 year

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