泛酸激酶2抗体

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英文名称  Anti-PANK2

中文名称  泛酸激酶2抗体

别    名  C20orf48; HARP; hPANK2; HSS; MGC15053; NBIA1; PANK2; PANK2_HUMAN; Pantothenate kinase 2 (Hallervorden Spatz syndrome); Pantothenate kinase 2; Pantothenic acid kinase 2; PKAN; RP23 387C21.4.

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

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

产品类型  一抗    

研究领域  肿瘤 细胞生物 免疫学 神经生物学 信号转导  

蛋白分子量  predicted molecular weight: 57kDa

性    状  Lyophilized or Liquid

泛酸激酶2抗体着国内抗体行业的崛起，我国自主品牌的抗体，都蛮多了，质量也不错，技术在逐渐成熟，价格也比国外的廉价的多，受到国内顾客的热捧，我们公司为了方便客户，特推出阴离子转运蛋白-1抗体规格15ug、30ug、50ug、100ug，15ug和30ug等，我们全国包邮，大中城市免费快递。抗体分子是生物学和医学领域用途最为广泛的蛋白分子。抗体作为疾病预防、诊断和治疗的制剂已有上百年的发展历史。随着生命科学研究的迅猛发展，抗体工程在生物技术领域越来越占有非常重要的地位。我公司可为您提供快速的、高质量的和经济的多克隆抗体制备服务，并将成为您在科研及生产中的得力助手。

免 疫 原  KLH conjugated synthetic peptide derived from human PANK2

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=1:50-200

（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 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.

Function : May be the master regulator of the CoA biosynthesis (By similarity).

Subunit :

Subcellular Location : Isoform 1: Mitochondrion.

Isoform 2: Cytoplasm (Potential).

Tissue Specificity : Ubiquitous.

DISEASE : Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1) [MIM:234200]; 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) [MIM:607236]. HARP is a rare syndrome with many clinical similarities to NBIA1.

泛酸激酶2抗体Similarity : Belongs to the type II pantothenate kinase family.

Database links : UniProtKB/Swiss-Prot: Q9BZ23.3