Quantity size:
0.2ml
Concentration:
1mg/ml泛酸激酶2抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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泛酸激酶2抗体 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泛酸激酶2抗体 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.
泛酸激酶2抗体Also known as:
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.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, .
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Immunogen: KLH conjugated synthetic peptide derived from human PANK2.
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泛酸激酶2抗体Predicted Molecular Weight: 57kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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企业名称
上海康朗生物科技有限公司
企业信息已认证
企业类型
信用代码
310112001510759
成立日期
2015-05-29
注册资本
100
经营范围
从事生物科技技术领域内技术开发、技术咨询、技术服务、技术转让,实验分析仪器、化工产品及原料(除危险化学品、监控化学品、烟花爆竹、民用爆竹物品、易制毒化学品)、仪器仪表、环保设备、酒店用品、皮革制品、床上用品、电子产品的销售。【依法须经批准的项目,经相关部门批准后方可开展经营活动】
上海康朗生物科技有限公司
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上海市闵行区闵北路88弄1-30号第22栋BV145室
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