Rabbit Anti-PANK2 antibody |
反应物种(预测) |
Rat,Dog,Pig,Cow,Horse |
产品应用(已验证) |
WB |
产品应用(可尝试) |
IHC,IF,ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:50-200, |
研究领域 |
肿瘤,细胞生物,免疫学,神经生物学,信号转导 |
标签 |
Array |
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Sample:
Bone (Mouse) Lysate at 40 ug
Primary: Anti-PANK2 (bs-8338R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 57 kD
Observed band size: 57 kD
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Sample:
Lane 1: HepG2 (Human) Cell Lysate at 30 ug
Lane 2: U251 (Human) Cell Lysate at 30 ug
Primary: Anti-PANK2 (bs-8338R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 62/50 kD
Observed band size: 62 kD
RRID:RRID
产品名称:Rabbit Anti-PANK2 antibody
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.
中文名称:泛酸激酶2抗体
英文名称:Rabbit Anti-PANK2 antibody
抗体来源: Rabbit
克隆类型:多克隆
细胞定位:细胞浆
性 状:Liquid
亚 型:IgG
纯化方法:affinity purified by Protein A
保存条件:Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
免 疫 原:KLH conjugated synthetic peptide derived from human PANK2
抗原表位:401-500/570
SWISS:Q9BZ23
Gene ID :80025
Human Gene ID:80025
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).
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 neurodeg
Similarity:Belongs to the type II pantothenate kinase family.
Important Note:This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.