ELK Biotechnology FKRP rabbit pAb
FKRP 단백질을 인식하는 토끼 다클론 항체로, Western blot, IF, ELISA에 적합. 인간, 마우스, 랫트 시료에 반응. 골지체 막 단백질 연구 및 근육 이영양증 관련 연구에 활용 가능. -20°C에서 1년 보관 가능.
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FKRP rabbit pAb
제품 개요
ELK Biotechnology의 FKRP rabbit pAb는 인간 FKRP(Fukutin-related protein)를 인식하는 토끼 다클론 항체입니다. 골지체 막 단백질로 알려진 FKRP는 근육 이영양증 및 관련 질환 연구에 중요한 표적 단백질입니다.
제품 정보
| 항목 | 내용 |
|---|---|
| Product name | FKRP rabbit pAb |
| Alternative Names | FKRP; Fukutin-related protein |
| Applications | WB; IF; ELISA |
| Recommended Dilutions | Western Blot: 1/500 - 1/2000 Immunofluorescence: 1/200 - 1/1000 ELISA: 1/20000 Not yet tested in other applications |
| Immunogen | The antiserum was produced against synthesized peptide derived from human FKRP (AA range: 1-50) |
| Host | Rabbit |
| Storage | -20°C / 1 year |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1 mg/ml |
| Observed Band | 50 kD |
| Gene ID (Human) | 79147 |
| Human Swiss-Prot No. | Q9H9S5 |
| Species Reactivity | Human; Mouse; Rat |
세포 내 위치
Golgi apparatus membrane; Single-pass type II membrane protein; Secreted; Cell membrane (sarcolemma); Rough endoplasmic reticulum; Cytoplasm.
According to some studies, the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted (PubMed:19900540).
Localization at the cell membrane may require the presence of dystroglycan (By similarity).
At the Golgi apparatus, localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization.
Detected in rough endoplasmic reticulum in myocytes (PubMed:17554798, PubMed:21886772).
Mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum (PubMed:15213246).
Background
This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan.
Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts.
Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.
[Provided by RefSeq, Oct 2008]
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