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维基百科

β2-微球蛋白

β2微球蛋白(英語:β2-microglobulin,缩写B2M)是MHC1類分子分子的组成部分之一,在所有有核的细胞中(即不包括红细胞)都有表达[6][7]。在人类基因组中,β2微球蛋白[8]B2M 基因所编码[7][9]

Β2微球蛋白
已知的結構
PDB直系同源搜索: PDBe RCSB
識別號
别名B2M;, entrez:567, IMD43, beta-2-microglobulin, Β2 microglobulin
外部IDOMIM:109700 MGI:88127 HomoloGene:2987 GeneCards:B2M
相關疾病
家族性腎澱粉樣變[1]
基因位置(人类
染色体15號染色體[2]
基因座15q21.1起始44,711,358 bp[2]
终止44,718,851 bp[2]
RNA表达模式


查阅更多表达数据
直系同源
物種人類小鼠
Entrez
Ensembl
UniProt
mRNA​序列

NM_004048

NM_009735

蛋白序列

NP_004039

NP_033865

基因位置​(UCSC)Chr 15: 44.71 – 44.72 MbChr 2: 121.98 – 121.98 Mb
PubMed​查找[4][5]
維基數據
檢視/編輯人類檢視/編輯小鼠

结构功能

 
β2微球蛋白在MHC Ⅰ类分子中的位置

β2-微球蛋白位于细胞表面的α3链旁,但与α链不同的是,β2并没有跨膜区域,且为非共价结合。在β2的“上方”(指远离细胞膜的一端)为α链的α1区和α2区,构成了MHC的肽结合区。

β2微球蛋白不止参与形成第一类MHC分子,还能组成与之类似的表面受体,如CD1Qa英语Alloantigen等。

β2微球蛋白还有一个功能,就是能与HFE蛋白英语HFE (gene)结合,来调控铁调素肝脏中的表达。鐵調素會與肠上皮细胞和巨噬细胞膜上的膜铁转运蛋白英语ferroportin作用。可以降低食物中和紅血球回收的鐵質攝取,该功能的丧失会导致铁过量和血色沉著病

临床意义

在长期血液透析的患者中,β2微球蛋白可以聚集成淀粉样纤维沉积在关节间隙,这种疾病被称为透析相关淀粉样变性

缺乏β2微球蛋白基因的小鼠模型已经被改造。这些小鼠证明β2微球蛋白对于MHC Ⅰ类分子的细胞表面表达和肽结合沟的稳定性是必需的。事实上,在没有β2微球蛋白的情况下,可以在表面检测到数量非常有限的MHC I类分子。在没有MHC I类分子的情况下,CD8 T细胞不能生长(CD8 T细胞是参与获得性免疫发展的T细胞的一个子集)。低水平的β2微球蛋白可显示HIV的非进展性。

多发性骨髓瘤淋巴瘤的β-2微球蛋白水平可能升高,但在这些情况下,原发性淀粉样变性(淀粉样轻链)和继发性淀粉样变性(淀粉样相关蛋白)更常见。β-2微球蛋白的正常值为<2 mg/L[10]。 然而,对于多发性骨髓瘤,β2微球蛋白的水平也可能处于谱的另一端。多发性骨髓瘤的诊断检测包括获取β2微球蛋白水平,因为该水平是一个重要的预后指标,水平<4 mg/L的患者的中位生存期预计为43个月,而水平>4 mg/L的患者的中位生存期仅为12个月[11]。β-2微球蛋白水平不能区分预后较好的不确定意义的单克隆免疫球蛋白血症英语Monoclonal gammopathy of undetermined significance(MGUS)和隐匿性(低度)骨髓瘤[12][13]

参考文献

  1. ^ 與Β2微球蛋白相關的疾病;在維基數據上查看/編輯參考. 
  2. ^ 2.0 2.1 2.2 GRCh38: Ensembl release 89: ENSG00000166710、​ENSG00000273686 - Ensembl, May 2017
  3. ^ 3.0 3.1 3.2 GRCm38: Ensembl release 89: ENSMUSG00000060802 - Ensembl, May 2017
  4. ^ Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
  5. ^ Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
  6. ^ B2M beta-2-microglobulin [Homo sapiens (human)]. NCBI. [2022-03-16] (英语). 
  7. ^ 7.0 7.1 Güssow, D.; Rein, R.; Ginjaar, I.; Hochstenbach, F.; Seemann, G.; Kottman, A.; Ploegh, H. L. The human beta 2-microglobulin gene. Primary structure and definition of the transcriptional unit. Journal of Immunology (Baltimore, Md.: 1950). 1987-11-01, 139 (9): 3132–3138 [2022-03-16]. ISSN 0022-1767. PMID 3312414. (原始内容于2022-01-05). 
  8. ^ Cunningham, Bruce A.; Wang, John L.; Berggard, Ingemar; Peterson, Per A. Complete amino acid sequence of β 2 -microglobulin. Biochemistry. 1973-11, 12 (24): 4811–4822 [2022-03-16]. ISSN 0006-2960. PMID 4586824. doi:10.1021/bi00748a001. (原始内容于2022-03-16) (英语). 
  9. ^ Suggs, S V; Wallace, R B; Hirose, T; Kawashima, E H; Itakura, K. Use of synthetic oligonucleotides as hybridization probes: isolation of cloned cDNA sequences for human beta 2-microglobulin.. Proceedings of the National Academy of Sciences. 1981-11, 78 (11): 6613–6617. ISSN 0027-8424. PMC 349099 . PMID 6171820. doi:10.1073/pnas.78.11.6613 (英语). 
  10. ^ Pignone M, Nicoll D; McPhee SJ. Pocket guide to diagnostic tests 4th. New York: McGraw-Hill. 2004: 191. ISBN 0-07-141184-4. 
  11. ^ Munshi NC, Longo DL, Anderson KC. Chapter 111: Plasma Cell Disorders. Loscalzo J, Longo DL, Fauci AS, Dennis LK, Hauser SL (编). Harrison's Principles of Internal Medicine 18th. McGraw-Hill Professional. 2011: 936–44. ISBN 0-07-174889-X. 
  12. ^ Rajkumar, S. Vincent. MGUS and Smoldering Multiple Myeloma: Update on Pathogenesis, Natural History, and Management. Hematology. 2005-01-01, 2005 (1): 340–345 [2022-03-16]. ISSN 1520-4391. doi:10.1182/asheducation-2005.1.340. (原始内容于2022-03-16) (英语). 
  13. ^ Bataille, R; Klein, B. Serum levels of beta 2 microglobulin and interleukin-6 to differentiate monoclonal gammopathy of undetermined significance [letter; comment]. Blood. 1992-11-01, 80 (9): 2433–2434. ISSN 0006-4971. doi:10.1182/blood.V80.9.2433.bloodjournal8092433 (英语). 

延伸阅读

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外部链接

微球蛋白, β2微球蛋白, 英語, microglobulin, 缩写b2m, 是mhc1類分子分子的组成部分之一, 在所有有核的细胞中, 即不包括红细胞, 都有表达, 在人类基因组中, β2微球蛋白, 由b2m, 基因所编码, Β2微球蛋白已知的結構pdb直系同源搜索, pdbe, rcsbpdbid列表1a1m, 1a1n, 1a1o, 1a6z, 1a9b, 1a9e, 1agb, 1agc, 1agd, 1age, 1agf, 1akj, 1ao7, 1b0g, 1b0r, 1bd2, 1c16, 1ce6. b2微球蛋白 英語 b2 microglobulin 缩写B2M 是MHC1類分子分子的组成部分之一 在所有有核的细胞中 即不包括红细胞 都有表达 6 7 在人类基因组中 b2微球蛋白 8 由B2M 基因所编码 7 9 B2微球蛋白已知的結構PDB直系同源搜索 PDBe RCSBPDBID列表1A1M 1A1N 1A1O 1A6Z 1A9B 1A9E 1AGB 1AGC 1AGD 1AGE 1AGF 1AKJ 1AO7 1B0G 1B0R 1BD2 1C16 1CE6 1DE4 1DUY 1DUZ 1E27 1E28 1EEY 1EEZ 1EFX 1EXU 1GZP 1GZQ 1HHG 1HHH 1HHI 1HHJ 1HHK 1HLA 1HSA 1HSB 1I1F 1I1Y 1I4F 1I7R 1I7T 1I7U 1IM3 1IM9 1JF1 1JGD 1JGE 1JHT 1JNJ 1K5N 1KPR 1KTL 1LDS 1LP9 1M05 1M6O 1MHE 1MI5 1OF2 1OGA 1OGT 1ONQ 1PY4 1Q94 1QEW 1QLF 1QQD 1QR1 1QRN 1QSE 1QSF 1QVO 1R3H 1S9W 1S9X 1S9Y 1SYS 1SYV 1TMC 1TVB 1TVH 1UQS 1UXS 1UXW 1VGK 1W0V 1W0W 1W72 1X7Q 1XH3 1XR8 1XR9 1XZ0 1YDP 1YPZ 1ZHK 1ZHL 1ZS8 1ZSD 1ZT4 1ZVS 2A83 2AK4 2AV7 2AXF 2AXG 2BCK 2BNQ 2BNR 2BSR 2BSS 2BST 2BVO 2BVP 2BVQ 2C7U 2CII 2CIK 2CLR 2D31 2D4D 2D4F 2DYP 2E8D 2ESV 2F53 2F54 2F74 2F8O 2GIT 2GJ6 2GT9 2GTW 2GTZ 2GUO 2H26 2H6P 2HJL 2HLA 2HN7 2J8U 2JCC 2NW3 2NX5 2P5E 2P5W 2PO6 2PYE 2RFX 2UWE 2V2W 2V2X 2VB5 2VLJ 2VLK 2VLL 2VLR 2X4N 2X4O 2X4P 2X4Q 2X4R 2X4S 2X4T 2X4U 2X70 2X89 2XKS 2XKU 2XPG 2YPK 2YPL 2YXF 2Z9T 3AM8 3B3I 3B6S 3BGM 3BH8 3BH9 3BHB 3BO8 3BP4 3BP7 3BVN 3BW9 3BWA 3BXN 3BZE 3BZF 3C9N 3CDG 3CII 3CIQ 3CZF 3D18 3D25 3D2U 3D39 3D3V 3DBX 3DHJ 3DHM 3DTX 3DXA 3EKC 3FFC 3FQN 3FQR 3FQT 3FQU 3FQW 3FQX 3FT2 3FT3 3FT4 3GIV 3GJF 3GSN 3GSO 3GSQ 3GSR 3GSU 3GSV 3GSW 3GSX 3H7B 3H9H 3H9S 3HAE 3HCV 3HG1 3HLA 3HPJ 3HUJ 3I6G 3I6K 3I6L 3IB4 3IXA 3JTS 3KLA 3KPO 3KPR 3KPS 3KWW 3KXF 3KYN 3KYO 3L3D 3L3G 3L3I 3L3J 3L3K 3LKN 3LKO 3LKP 3LKQ 3LKR 3LKS 3LN4 3LN5 3LOW 3LV3 3M17 3M1B 3MGO 3MGT 3MR9 3MRB 3MRC 3MRD 3MRE 3MRF 3MRG 3MRH 3MRI 3MRJ 3MRK 3MRL 3MRM 3MRN 3MRO 3MRP 3MRQ 3MRR 3MV7 3MV8 3MV9 3MYJ 3MYZ 3MZT 3NA4 3NFN 3O3A 3O3B 3O3D 3O3E 3O4L 3OX8 3OXR 3OXS 3PWJ 3PWL 3PWN 3PWP 3QDA 3QDG 3QDJ 3QDM 3QEQ 3QFD 3QFJ 3QZW 3RL1 3RWJ 3S6C 3SDX 3SJV 3SKM 3SKO 3SPV 3T8X 3TID 3TIE 3TLR 3TM6 3TO2 3TZV 3U0P 3UPR 3UTQ 3UTS 3UTT 3V5D 3V5H 3V5K 3VCL 3VFR 3VFS 3VFT 3VFU 3VFV 3VFW 3VH8 3VRI 3VRJ 3VWJ 3VWK 3W39 4E0K 4E0L 4E5X 4EN3 4EUP 4F7M 4F7P 4F7T 4FTV 4FXL 4G8G 4G8I 4G9D 4G9F 4GKS 4GUP 4HKJ 4I4W 4JFD 4JFE 4JFF 4JFO 4JFP 4JFQ 4JQX 4JRX 4JRY 4K7F 4KDT 4L4T 4L4V 1CG9 1N2R 1P7Q 1S8D 1T1W 1T1X 1T1Y 1T1Z 1T20 1T21 1T22 2AV1 2FYY 2FZ3 2HJK 3DX6 3DX7 3DX8 3KPL 3KPM 3KPN 3KPP 3KPQ 3LOZ 3OV6 3REW 3RL2 3VXM 3VXN 3VXO 3VXP 3VXR 3VXS 3VXU 3W0W 3WL9 3WLB 3WUW 3X11 3X12 3X13 3X14 4GKN 4HWZ 4HX1 4I48 4K71 4L29 4L3C 4L3E 4LCW 4LCY 4LHU 4LNR 4M8V 4MJ5 4MJ6 4MJI 4MNQ 4N0F 4N0U 4N8V 4NNX 4NNY 4NO0 4NO2 4NO3 4NO5 4NQC 4NQD 4NQE 4NQV 4NQX 4NT6 4O2C 4O2E 4O2F 4ONO 4PJ5 4PJ7 4PJ8 4PJ9 4PJA 4PJB 4PJC 4PJD 4PJE 4PJF 4PJG 4PJH 4PJI 4PJX 4PR5 4PRA 4PRB 4PRD 4PRE 4PRH 4PRI 4PRN 4PRP 4QOK 4QRP 4QRQ 4QRR 4QRS 4QRT 4QRU 4U1H 4U1I 4U1J 4U1K 4U1L 4U1M 4U1N 4U1S 4U6X 4U6Y 4UQ2 4UQ3 4WJ5 4WO4 4WU5 4WU7 4X6C 4X6D 4X6E 4X6F 4XXC 4WDI 4Z76 4Z77 4Z78 4R9H 4RA3 4RAH 5D2L 5D2N 4WW2 5DEG 4RMT 5DEF 4RMW 4RMV 4RMR 5D7J 4RMQ 4RMS 4WWK 5BXF 5D5M 5D7L 4RMU 4WUU 5D7I 5EU5 5EO0 5EO1 5EU4 5BRZ 5EU6 5BS0 5EU3 5C9J 5D9S 5HGA 5C0D 5C0B 5C0I 5C0A 5B38 5E9D 5B39 5C0H 5HHN 5HGD 5C08 5CFH 5CKG 4ZEZ 5C0C 5HGH 5C0J 5HHQ 5DDH 5HHM 5HGB 5C09 5HYJ 5CKA 5C07 5HHP 5C0G 5C0F 5HHO 5C0E識別號别名B2M entrez 567 IMD43 beta 2 microglobulin B2 microglobulin外部IDOMIM 109700 MGI 88127 HomoloGene 2987 GeneCards B2M相關疾病家族性腎澱粉樣變 1 基因位置 人类 染色体15號染色體 2 基因座15q21 1起始44 711 358 bp 2 终止44 718 851 bp 2 基因位置 小鼠 染色体小鼠2号染色体 3 基因座2 E5 2 60 55 cM起始121 978 167 bp 3 终止121 983 564 bp 3 RNA表达模式查阅更多表达数据基因本體分子功能 血浆蛋白结合 相同蛋白质结合細胞組分 HFE transferrin receptor complex 高尔基体 phagocytic vesicle membrane early endosome membrane endoplasmic reticulum lumen 膜 粘着斑 Golgi membrane 细胞膜 細胞外區域 MHC class I protein complex ER to Golgi transport vesicle membrane early endosome lumen 外排體 external side of plasma membrane 細胞外空間 细胞质基质 cell surface specific granule lumen recycling endosome membrane tertiary granule lumen MHC class I peptide loading complex生物學過程 positive regulation of receptor mediated endocytosis cellular response to iron ion response to cadmium ion negative regulation of receptor binding negative regulation of neuron projection development antigen processing and presentation of exogenous peptide antigen via MHC class I TAP dependent T cell differentiation in thymus immune system process interferon gamma mediated signaling pathway antigen processing and presentation of peptide antigen via MHC class I iron ion homeostasis positive regulation of T cell mediated cytotoxicity mitigation of host defenses by virus antigen processing and presentation of exogenous peptide antigen via MHC class I TAP independent regulation of membrane depolarization antigen processing and presentation of endogenous peptide antigen via MHC class I positive regulation of ferrous iron binding positive regulation of transferrin receptor binding retina homeostasis positive regulation of T cell cytokine production protein refolding 免疫反应 regulation of immune response positive regulation of receptor binding positive regulation of protein binding 先天免疫系統 cellular response to lipopolysaccharide response to molecule of bacterial origin antigen processing and presentation of exogenous protein antigen via MHC class Ib TAP dependent iron ion transport neutrophil degranulation regulation of erythrocyte differentiation cellular response to iron III ion antimicrobial humoral immune response mediated by antimicrobial peptide positive regulation of iron ion import across plasma membrane regulation of iron ion transportSources Amigo QuickGO直系同源物種人類小鼠Entrez56712010EnsemblENSG00000166710 ENSG00000273686ENSMUSG00000060802UniProtP61769P01887mRNA 序列NM 004048NM 009735蛋白序列NP 004039NP 033865基因位置 UCSC Chr 15 44 71 44 72 MbChr 2 121 98 121 98 MbPubMed 查找 4 5 維基數據檢視 編輯人類檢視 編輯小鼠 目录 1 结构功能 2 临床意义 3 参考文献 4 延伸阅读 5 外部链接结构功能 编辑 b2微球蛋白在MHC 类分子中的位置 b2 微球蛋白位于细胞表面的a3链旁 但与a链不同的是 b2并没有跨膜区域 且为非共价结合 在b2的 上方 指远离细胞膜的一端 为a链的a1区和a2区 构成了MHC的肽结合区 b2微球蛋白不止参与形成第一类MHC分子 还能组成与之类似的表面受体 如CD1和Qa 英语 Alloantigen 等 b2微球蛋白还有一个功能 就是能与HFE蛋白 英语 HFE gene 结合 来调控铁调素在肝脏中的表达 鐵調素會與肠上皮细胞和巨噬细胞膜上的膜铁转运蛋白 英语 ferroportin 作用 可以降低食物中和紅血球回收的鐵質攝取 该功能的丧失会导致铁过量和血色沉著病 临床意义 编辑在长期血液透析的患者中 b2微球蛋白可以聚集成淀粉样纤维沉积在关节间隙 这种疾病被称为透析相关淀粉样变性 缺乏b2微球蛋白基因的小鼠模型已经被改造 这些小鼠证明b2微球蛋白对于MHC 类分子的细胞表面表达和肽结合沟的稳定性是必需的 事实上 在没有b2微球蛋白的情况下 可以在表面检测到数量非常有限的MHC I类分子 在没有MHC I类分子的情况下 CD8 T细胞不能生长 CD8 T细胞是参与获得性免疫发展的T细胞的一个子集 低水平的b2微球蛋白可显示HIV的非进展性 多发性骨髓瘤和淋巴瘤的b 2微球蛋白水平可能升高 但在这些情况下 原发性淀粉样变性 淀粉样轻链 和继发性淀粉样变性 淀粉样相关蛋白 更常见 b 2微球蛋白的正常值为 lt 2 mg L 10 然而 对于多发性骨髓瘤 b2微球蛋白的水平也可能处于谱的另一端 多发性骨髓瘤的诊断检测包括获取b2微球蛋白水平 因为该水平是一个重要的预后指标 水平 lt 4 mg L的患者的中位生存期预计为43个月 而水平 gt 4 mg L的患者的中位生存期仅为12个月 11 b 2微球蛋白水平不能区分预后较好的不确定意义的单克隆免疫球蛋白血症 英语 Monoclonal gammopathy of undetermined significance MGUS 和隐匿性 低度 骨髓瘤 12 13 参考文献 编辑 與B2微球蛋白相關的疾病 在維基數據上查看 編輯參考 2 0 2 1 2 2 GRCh38 Ensembl release 89 ENSG00000166710 ENSG00000273686 Ensembl May 2017 3 0 3 1 3 2 GRCm38 Ensembl release 89 ENSMUSG00000060802 Ensembl May 2017 Human PubMed Reference 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引文格式1维护 PMC格式 link 外部链接 编辑醫學主題詞表 MeSH beta 2 Microglobulin 取自 https zh wikipedia org w index php title B2 微球蛋白 amp oldid 71043007, 维基百科,wiki,书籍,书籍,图书馆,

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