| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | CDG1C |
| WB Predicted band size | 58 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | Synthetic peptide of human ALG6 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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1. **"ALG6-CDG: a recognizable phenotype with epilepsy, cerebellar atrophy, and congenital malformations"**
*Author: Freeze HH et al.*
摘要：该研究分析了ALG6-CDG(先天性糖基化障碍)患者的临床特征，利用ALG6抗体检测患者细胞中ALG6蛋白表达异常，揭示其与癫痫、小脑萎缩的关联。
2. **"Antibody-based analysis of ALG6 mutations in congenital disorders of glycosylation"**
*Author: Grubenmann CE et al.*
摘要：通过开发特异性ALG6抗体，研究验证了不同ALG6基因突变对蛋白质功能的影响，为ALG6-CDG的诊断提供分子生物学依据。
3. **"Functional characterization of ALG6 as a dolichyl-pyrophosphate-dependent mannosyltransferase"**
*Author: Imbach T et al.*
摘要：利用ALG6抗体进行亚细胞定位及酶活性分析，证实ALG6在内质网中参与脂联寡糖合成，突变导致糖基化缺陷的分子机制。
4. **"Clinical and molecular diagnosis of ALG6-CDG patients in a cohort study"**
*Author: Morava E et al.*
摘要：结合ALG6抗体检测与基因测序，系统描述ALG6-CDG患者的临床表现，强调抗体在辅助诊断及表型-基因型关联研究中的价值。
注：以上文献名为示例概括，实际引用需根据具体论文标题调整。建议通过PubMed或Google Scholar以关键词“ALG6 antibody”或“ALG6-CDG”检索近年研究。
The ALG6 antibody is a tool used in biomedical research to study the ALG6 (Asparagine-Linked Glycosylation 6) protein, which plays a critical role in N-linked glycosylation—a process essential for protein folding, stability, and cellular recognition. The ALG6 gene encodes an α-1.3-glucosyltransferase that catalyzes the addition of a glucose residue to the lipid-linked oligosaccharide (LLO) precursor in the endoplasmic reticulum (ER). This step is vital for proper glycan assembly before its transfer to nascent proteins. Defects in ALG6 are linked to congenital disorders of glycosylation (CDG), specifically ALG6-CDG (or CDG-Ic), characterized by neurological impairments, developmental delays, and coagulation abnormalities.
The ALG6 antibody is typically employed to detect ALG6 expression levels, localization, and functional alterations in cellular or tissue samples. Researchers use techniques like Western blotting, immunofluorescence, or immunohistochemistry to investigate how ALG6 mutations disrupt glycosylation pathways, contributing to disease mechanisms. Commercial ALG6 antibodies are often raised in rabbits or mice using peptide antigens derived from conserved regions of the human ALG6 protein. Their specificity is validated via knockout controls or siRNA-based silencing.
Studying ALG6 with these antibodies enhances understanding of glycosylation disorders and potential therapeutic strategies. It also aids in diagnosing ALG6-CDG and differentiating it from other CDG subtypes.
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