产品说明
一般描述
This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS). (provided by RefSeq)
免疫原
PEX12 (AAH31085, 1 a.a. ~ 359 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MAEHGAHFTAASVADDQPSIFEVVAQDSLMTAVRPALQHVVKVLAESNPTHYGFLWRWFDEIFTLLDLLLQQHYLSRTSASFSENFYGLKRIVMGDTHKSQRLASAGLPKQQLWKSIMFLVLLPYLKVKLEKLVSSLREEDEYSIHPPSSRWKRFYRAFLAAYPFVNMAWEGWFLVQQLRYILGKAQHHSPLLRLAGVQLGRLTVQDIQALEHKPAKASMMQQPARSVSEKINSALKKAVGGVALSLSTGLSVGVFFLQFLDWWYSSENQETIKSLTALPTPPPPVHLDYNSDSPLLPKMKTVCPLCRKTRVNDTVLATSGYVFCYRCVFHYVRSHQACPITGYPTEVQHLIKLYSPEN
外形
Solution in phosphate buffered saline, pH 7.4
法律信息
GenBank is a registered trademark of United States Department of Health and Human Services
基本信息
NACRES | NA.41 |
产品性质
质量水平 | 100 |
生物来源 | mouse |
偶联物 | unconjugated |
抗体形式 | purified immunoglobulin |
antibody product type | primary antibodies |
克隆 | 2G6, monoclonal |
形式 | buffered aqueous solution |
species reactivity | human |
technique(s) | indirect ELISA: suitable |
同位素/亚型 | IgG2aκ |
GenBank® accession no. | BC031085 |
UniProt登记号 | O00623 |
运输 | dry ice |
储存温度 | −20℃ |
Gene Information | human ... PEX12(5193) |
安全信息
储存分类代码 | 12 - Non Combustible Liquids |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
个人防护装备 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
Sigma-Aldrich