VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures.VLDLR-CH is inherited in an autosomal recessive manner. Carrier testing for at-risk relatives, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible when the pathogenic variants in a family are known.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
100 μg | ¥ 1,920 | 5日内发货 | ||
500 μg | ¥ 7,710 | 5日内发货 | ||
1 mg | ¥ 12,800 | 5日内发货 |
生物活性 | Mouse VLDLR, His Tag immobilized on CM5 Chip can bind Mouse PCSK9, His Tag with an affinity constant of 0.28 nM as determined in SPR assay. |
产品描述 | VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures.VLDLR-CH is inherited in an autosomal recessive manner. Carrier testing for at-risk relatives, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible when the pathogenic variants in a family are known. |
种属 | Mouse |
表达系统 | HEK293 |
标签 | C-His |
蛋白编号 | P98156 |
别名 | CHRMQ1, VLDLR, FLJ35024, VLDLRCH, RP11-320E16.1, VLDL-R |
蛋白构建 | Recombinant Mouse VLDLR Protein is expressed from HEK293 with His tag at the C-Terminus. It contains Gly28-Ser797.[Accession |P98156] |
蛋白纯度 | > 95% as determined by Tris-Bis PAGE |
分子量 | The protein has a predicted MW of 86.1 kDa. Due to glycosylation, the protein migrates to 110-130 kDa based on Tris-Bis PAGE result. |
内毒素 | Less than 1EU per μg by the LAL method. |
缓冲液 | Lyophilized from 0.22μm filtered solution in PBS (pH 7.4). Normally 8% trehalose is added as protectant before lyophilization. |
复溶方法 | Centrifuge the tube before opening. Reconstituting to a concentration more than 100 μg/ml is recommended. Dissolve the lyophilized protein in distilled water. |
存储 |
-20 to -80°C for 12 months as supplied from date of receipt. -20 to -80°C for 3-6 months in unopened state after reconstitution. 2-8°C for 2-7 days after reconstitution. Recommend to aliquot the protein into smaller quantities for optimal storage. Please minimize freeze-thaw cycles. |
运输方式 |
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise. |
研究背景 | VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures.VLDLR-CH is inherited in an autosomal recessive manner. Carrier testing for at-risk relatives, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible when the pathogenic variants in a family are known. |
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VLDLR Protein, Mouse, Recombinant (His) CHRMQ1 VLDLR FLJ35024 VLDLRCH CHRMQ-1 RP11-320E16.1 FLJ-35024 VLDL-R FLJ 35024 CHRMQ 1 recombinant recombinant-proteins proteins protein