| 产品: | FKRP 抗体 |
| 货号: | DF3438 |
| 描述: | Rabbit polyclonal antibody to FKRP |
| 应用: | WB IF/ICC |
| 反应: | Human, Mouse, Rat |
| 预测: | Pig, Dog |
| 分子量: | 50 KD; 55kD(Calculated). |
| 蛋白号: | Q9H9S5 |
| RRID: | AB_2835804 |
产品描述
*The optimal dilutions should be determined by the end user.
*Tips:
WB: 适用于变性蛋白样本的免疫印迹检测. IHC: 适用于组织样本的石蜡(IHC-p)或冰冻(IHC-f)切片样本的免疫组化/荧光检测. IF/ICC: 适用于细胞样本的荧光检测. ELISA(peptide): 适用于抗原肽的ELISA检测.
引用格式: Affinity Biosciences Cat# DF3438, RRID:AB_2835804.
展开/折叠
Fkrp; FKRP_HUMAN; FLJ12576; Fukutin related protein; Fukutin-related protein; LGMD2I; MDC1C; MGC2991;
抗原和靶标
Expressed in the retina (at protein level) (PubMed:29416295). Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver, kidney, and pancreas (PubMed:11592034).
- Q9H9S5 FKRP_HUMAN:
- Protein BLAST With
- NCBI/
- ExPASy/
- Uniprot
MRLTRCQAALAAAITLNLLVLFYVSWLQHQPRNSRARGPRRASAAGPRVTVLVREFEAFDNAVPELVDSFLQQDPAQPVVVAADTLPYPPLALPRIPNVRLALLQPALDRPAAASRPETYVATEFVALVPDGARAEAPGLLERMVEALRAGSARLVAAPVATANPARCLALNVSLREWTARYGAAPAAPRCDALDGDAVVLLRARDLFNLSAPLARPVGTSLFLQTALRGWAVQLLDLTFAAARQPPLATAHARWKAEREGRARRAALLRALGIRLVSWEGGRLEWFGCNKETTRCFGTVVGDTPAYLYEERWTPPCCLRALRETARYVVGVLEAAGVRYWLEGGSLLGAARHGDIIPWDYDVDLGIYLEDVGNCEQLRGAEAGSVVDERGFVWEKAVEGDFFRVQYSESNHLHVDLWPFYPRNGVMTKDTWLDHRQDVEFPEHFLQPLVPLPFAGFVAQAPNNYRRFLELKFGPGVIENPQYPNPALLSLTGSG
种属预测
score>80的预测可信度较高，可尝试用于WB检测。*预测模型主要基于免疫原序列比对，结果仅作参考，不作为质保凭据。
High(score>80) Medium(80>score>50) Low(score<50) No confidence
翻译修饰 - Q9H9S5 作为底物
| Site | PTM Type | Enzyme | Source |
|---|---|---|---|
| T162 | Phosphorylation | Uniprot | |
| N172 | N-Glycosylation | Uniprot | |
| S174 | Phosphorylation | Uniprot | |
| N209 | N-Glycosylation | Uniprot | |
| K396 | Ubiquitination | Uniprot |
研究背景
Catalyzes the transfer of CDP-ribitol to ribitol 5-phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1.
N-glycosylated.
Golgi apparatus membrane>Single-pass type II membrane protein. Secreted. Cell membrane>Sarcolemma. Rough endoplasmic reticulum. Cytoplasm.
Note: 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). In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum (PubMed:15213246).
Expressed in the retina (at protein level). Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver, kidney, and pancreas.
Homodimer; disulfide-linked. Forms a complex composed of FKRP, FKTN/fukutin, and RXYLT1/TMEM5. Exists also as large multimeric protein complexes. May interact with the dystrophin-glycoprotein complex (DGC) (By similarity).
Belongs to the LicD transferase family.
研究领域
· Metabolism > Global and overview maps > Metabolic pathways.
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