GCSH 抗体 (AA 101-173) (AbBy Fluor® 680)
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- 抗原 See all GCSH 抗体
- GCSH (Glycine Cleavage System H Protein (GCSH))
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抗原表位
- AA 101-173
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适用
- 人
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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 GCSH antibody is conjugated to AbBy Fluor® 680
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 预测反应
- Human,Mouse,Rat,Horse
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human GCSH
- 亚型
- IgG
- Top Product
- Discover our top product GCSH Primary Antibody
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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
- GCSH (Glycine Cleavage System H Protein (GCSH))
- 别名
- GCSH (GCSH 产品)
- 别名
- GCE antibody, NKH antibody, GCSH antibody, H-PROTEIN antibody, 1100001L02Rik antibody, 5730591C18Rik antibody, wu:fb14d10 antibody, wu:fw84b08 antibody, zgc:112535 antibody, BA5228 antibody, gce antibody, gcsh antibody, nkh antibody, wu:fd56h06 antibody, zgc:110557 antibody, zgc:92732 antibody, glycine cleavage system protein H antibody, glycine cleavage system protein H (aminomethyl carrier) antibody, glycine cleavage system lipoyl carrier protein GcvH antibody, glycine cleavage system H protein antibody, glycine cleavage system protein H (aminomethyl carrier), a antibody, glycine cleavage system carrier of aminomethyl moiety GcvH antibody, leucine rich repeat containing 34 antibody, glycine cleavage system protein H (aminomethyl carrier) L homeolog antibody, glycine cleavage system protein H (aminomethyl carrier), b antibody, GCSH antibody, Gcsh antibody, gcvH-2 antibody, gcvH antibody, gcsha antibody, LRRC34 antibody, gcsh.L antibody, gcshb antibody
- 背景
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Synonyms: GCE, GCSH, GCSH_HUMAN, Glycine cleavage system H protein, Glycine cleavage system H protein mitochondrial, Glycine cleavage system protein H aminomethyl carrier, Glycine cleavage system protein H, Lipoic acid containing protein, mitochondrial, Mitochondrial glycine cleavage system H protein, NKH.
Background: GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3 % of the human genome.
- 基因ID
- 2653
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