PANK2 抗体 (AA 401-500) (AbBy Fluor® 555)
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- 抗原 See all PANK2 抗体
- PANK2 (Pantothenate Kinase 2 (PANK2))
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抗原表位
- AA 401-500
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适用
- 小鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This PANK2 antibody is conjugated to AbBy Fluor® 555
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 小鼠
- 预测反应
- Human,Rat,Dog,Cow,Pig,Horse
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human PANK2
- 亚型
- IgG
- Top Product
- Discover our top product PANK2 Primary Antibody
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anti-Pantothenate Kinase 2 (PANK2) antibody
PANK2 适用: 人, 大鼠, 犬, 猴 WB, IHC, IF, IHC (p), FACS 宿主: 小鼠 Monoclonal 3H9 unconjugated
anti-Pantothenate Kinase 2 (PANK2) (AA 437-490) antibodyPANK2 适用: 人 WB, ELISA, IHC, IF 宿主: 兔 Polyclonal unconjugated
anti-Pantothenate Kinase 2 (PANK2) (AA 205-260) antibodyPANK2 适用: 人 WB, ELISA, IHC (p) 宿主: 小鼠 Monoclonal 2B12 unconjugated
anti-Pantothenate Kinase 2 (PANK2) (N-Term) antibodyPANK2 适用: 人 WB, ELISA, IF, ICC 宿主: 兔 Polyclonal unconjugated
anti-Pantothenate Kinase 2 (PANK2) (AA 459-508) antibodyPANK2 适用: 人, 大鼠, 犬, Cow, 马, 绵羊, Pig, Bat WB 宿主: 兔 Polyclonal unconjugated
anti-Pantothenate Kinase 2 (PANK2) (AA 300-400) antibodyPANK2 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Pantothenate Kinase 2 (PANK2) antibodyPANK2 适用: 人 WB, IF, FACS 宿主: 小鼠 Monoclonal 3G4 unconjugated
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- 应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
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- 抗原
- PANK2 (Pantothenate Kinase 2 (PANK2))
- 别名
- PANK2 (PANK2 产品)
- 别名
- ATPANK2 antibody, F10M6.180 antibody, F10M6_180 antibody, pantothenate kinase 2 antibody, PANK2 antibody, C20orf48 antibody, HARP antibody, HSS antibody, NBIA1 antibody, PKAN antibody, 4933409I19Rik antibody, AI642621 antibody, pantothenate kinase 2 antibody, pantothenate kinase 2, mitochondrial antibody, ribonuclease A family member 11 (inactive) antibody, PANK2 antibody, Tsp_01576 antibody, LOC100539295 antibody, Pank2 antibody, RNASE11 antibody
- 背景
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Synonyms: Pantothenate kinase 2, mitochondrial, hPanK2, Pantothenic acid kinase 2, PANK2, C20orf48, PANK2_HUMAN
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 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.
- 基因ID
- 80025
- UniProt
- Q9BZ23
- 途径
- Ribonucleoside Biosynthetic Process
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