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激光干涉仪型号： XL80 -
Hitachi日立CT15RE台式离心机型号： CT15RE -
Hitachi日立超速离心机CP70ME型号： CP70ME -
电热鼓风干燥箱型号： CS101-ABN -
Agilent 8890 气相色谱系统型号： 8890 GC -
全自动桌面溶出度测试系统RTJ3000+DS-3000型号： RT-J3000+DS3000 -
全自动溶出度测试仪RT-3型号： RT-3 -
流通池法溶出度测试仪DF-7型号： DF-7 -
自动桌面溶出度测试系统RT-J2000+DS2000α/DS3000型号： RT-J2000+DS2000α/DS3000 -
日立Primaide PLUS高效液相色谱仪型号： Primaide PLUS
获取电话Quantity size:
0.2ml
Concentration:
1mg/ml甘氨酸裂解系统H蛋白抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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 甘氨酸裂解系统H蛋白抗体(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.
甘氨酸裂解系统H蛋白抗体Also known as:
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.
Specificity:
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Rabbit Polyclonal IgG,甘氨酸裂解系统H蛋白抗体 affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Horse, .
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Immunogen: KLH conjugated synthetic peptide derived from human GCSH.
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Predicted Molecular Weight: 14kDa.
甘氨酸裂解系统H蛋白抗体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 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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