| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20-1/50 | 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 | 咨询技术 | Human,Mouse,Rat |
| Aliases | 3H3M; HMDH; HMG CoA reductase; HMG CoAR;;HMGCR |
| WB Predicted band size | 97 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 | A synthesized peptide derived from human HMGCR |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于HMGCR抗体的3篇参考文献及其摘要概括：
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1. **文献名称**：*Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy*
**作者**：Mammen AL, Chung T, Christopher-Stine L, et al.
**摘要**：该研究首次报道了他汀类药物使用与HMGCR抗体阳性自身免疫性肌病(IMNM)的关联。通过检测患者血清中的抗体，发现抗体水平与他汀暴露史及肌坏死病理特征相关，提示其作为诊断标志物的潜在价值。
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2. **文献名称**：*HMG-CoA reductase antibody-associated necrotizing myopathy: A systematic review*
**作者**：Limaye V, Bundell C, Hollingsworth P, et al.
**摘要**：系统综述分析了HMGCR抗体阳性患者的临床特征，包括肌无力、肌酸激酶升高及对他汀停药后的治疗反应。研究强调早期免疫治疗(如糖皮质激素、利妥昔单抗)对改善预后的重要性。
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3. **文献名称**：*Statin-associated autoimmune myopathy: A distinct clinical and pathological entity*
**作者**：Christopher-Stine L, Casciola-Rosen LA, Hong G, et al.
**摘要**：本研究描述了HMGCR抗体阳性肌病的独特病理表现，如肌纤维坏死和MHC-I上调，并指出即使停用他汀后，患者仍需长期免疫抑制治疗以控制疾病进展。
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以上文献均发表于《Arthritis & Rheumatology》等权威期刊，聚焦HMGCR抗体的病理机制、诊断及临床管理。如需具体年份或卷期号，可进一步补充。
HMGCR (3-hydroxy-3-methylglutaryl-coenzyme A reductase) antibodies are autoantibodies associated with immune-mediated necrotizing myopathy (IMNM), a subtype of idiopathic inflammatory myopathy. HMGCR is the rate-limiting enzyme in cholesterol biosynthesis and the pharmacological target of statins. Anti-HMGCR antibodies were first identified in 2010. primarily in patients with statin-associated autoimmune myopathy, though they also occur in statin-naïve individuals, particularly children. These IgG autoantibodies are detected in approximately 6% of adult myositis patients and 30-50% of IMNM cases.
The pathogenesis involves statin-triggered immune responses in genetically susceptible individuals (e.g., HLA-DRB1*11:01 carriers). Statins upregulate HMGCR expression in muscle, potentially exposing cryptic epitopes and driving autoantibody production. Anti-HMGCR antibodies may directly inhibit enzyme activity or promote complement-mediated cytotoxicity, contributing to muscle fiber necrosis.
Clinically, patients present with progressive proximal weakness, elevated creatine kinase (>10x normal), and necrotic muscle biopsies. Diagnosis combines serological testing (immunoprecipitation/ELISA) with clinical features. Treatment involves immunosuppression (corticosteroids, IVIG, rituximab) and statin discontinuation when applicable. Antibody levels often correlate with disease activity, aiding therapeutic monitoring. Research continues to clarify their exact pathogenic role and optimize management strategies.
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