FUCA1 Protein|FUCA1蛋白质|现货|报价

FUCA1蛋白产品信息

别名: FUCA1
表达方式: 
A DNA sequence encoding the human FUCA1 (P04066) (Gln32-Lys466) was expressed with a polyhistidine tag at the C-terminus.
种属: Human
表达宿主: Human Cells

FUCA1 Protein QC Testing

纯度: > 95% as determined by SDS-PAGE SDS-PAGE
内**: < 1.0 EU per μg of the protein as determined by the LAL method
稳定性: Samples are stable for up to twelve months from date of receipt at -70℃
预测N端: Gln 32
分子量: 
The recombinant human FUCA1 consists of  446 amino acids and predicts a molecular mass of 51.9 KDa. It migrates as an approximately 57 KDa band in SDS-PAGE under reducing conditions.
缓冲液: Lyophilized from sterile PBS, pH 7.4.
Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Please contact us for any concerns or special requirements.

FUCA1 Protein Usage Guide

储存方法: Store it under sterile conditions at -70℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

FUCA1蛋白背景综述

FUCA1 is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. Mutations in FUCA1 gene can cause fucosidosis. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective FUCA1 with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form; coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form; and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form.

参考文献

Yang M. et al., 1993, Biochem Biophys Res Commun. 189 (2): 1063-8.
Fukushima H. et al., 1991, J Inherit Metab Dis. 13 (5): 761-5.
Kretz KA. et al., 1990, J Mol Neurosci. 1 (3): 177-80.

规格：10ug

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