| 產(chǎn)品編號(hào) | bs-8338R |
| 英文名稱 | PANK2 Rabbit pAb |
| 中文名稱 | 泛酸激酶2抗體 |
| 別 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. |
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Specific References (1) | bs-8338R has been referenced in 1 publications.
[IF=9.918] Jie Hao. et al. Multifunctional miR181a nanoparticles promote highly efficient radiotherapy for rectal cancer. MATER TODAY ADV. 2022 Dec;16:100317 WB ; Mouse.
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| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | Human,Mouse (predicted: Rat,Pig,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 57 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PANK2: 401-500/570 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 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. Similarity: Belongs to the type II pantothenate kinase family. SWISS: Q9BZ23 Gene ID: 80025 Database links: Entrez Gene: 80025 Human Omim: 606157 Human SwissProt: Q9BZ23 Human Unigene: 516859 Human |
| 產(chǎn)品圖片 |
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
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
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