Β2微球蛋白

Beta-2-microglobulin; β-2-微球蛋白
	; β2微球蛋白在I類MHC中的位置
有效結構
PDB	直系同源檢索：PDBe, RCSB
PDB查詢代碼列表
	1A1M, 1A1N, 1A1O, 1A6Z, 1A9B, 1A9E, 1AGB, 1AGC, 1AGD, 1AGE, 1AGF, 1AKJ, 1AO7, 1B0G, 1B0R, 1BD2, 1C16, 1CE6, 1CG9, 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, 1N2R, 1OF2, 1OGA, 1OGT, 1ONQ, 1P7Q, 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, 2AV1, 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, 2FYY, 2FZ3, 2GIT, 2GJ6, 2GT9, 2GTW, 2GTZ, 2GUO, 2H26, 2H6P, 2HJK, 2HJL, 2HLA, 2HN7, 2J8U, 2JCC, 2NW3, 2NX5, 2P5E, 2P5W, 2PO6, 2PYE, 2RFX, 2UWE, 2V2W, 2V2X, 2VB5, 2VLJ, 2VLK, 2VLL, 2VLR, 2X4N, 2X4O, 2X4P, 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, 3DX6, 3DX7, 3DX8, 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, 3KPL, 3KPM, 3KPN, 3KPO, 3KPP, 3KPQ, 3KPR, 3KPS, 3KWW, 3KXF, 3KYN, 3KYO, 3L3D, 3L3G, 3L3I, 3L3J, 3L3K, 3LKN, 3LKO, 3LKP, 3LKQ, 3LKR, 3LKS, 3LN4, 3LN5, 3LOW, 3LOZ, 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, 3OV6, 3OX8, 3OXR, 3OXS, 3PWJ, 3PWL, 3PWN, 3PWP, 3QDA, 3QDG, 3QDJ, 3QDM, 3QEQ, 3QFD, 3QFJ, 3QZW, 3REW, 3RL1, 3RL2, 3RWJ, 3S6C, 3SDX, 3SJV, 3SKM, 3SKO, 3SPV, 3T8X, 3TID, 3TIE, 3TLR, 3TM6, 3TO2, 3TZV, 3U0P, 3UPR, 3UTQ, 3UTS, 3UTT, 3V5D, 3V5H, 3V5K, 3VCL, 3VFM, 3VFS, 3VFU, 3VFV, 3VFW, 3VH8, 3VRI, 3VRJ, 3VWJ, 3VWK, 3VXM, 3VXN, 3VXO, 3VXP, 3VXR, 3VXS, 3VXU, 3W0W, 3W39, 4E0K, 4E0L, 4E5X, 4EN3, 4EUP, 4EUQ, 4F7M, 4F7P, 4F7T, 4FTV, 4FXL, 4G8G, 4G8I, 4G9D, 4G9F, 4GKN, 4GKS, 4GUP, 4HKJ, 4HWZ, 4HX1, 4I48, 4I4W, 4JFD, 4JFE, 4JFF, 4JFO, 4JFP, 4JFQ, 4JQX, 4JRX, 4JRY, 4K71, 4K7F, 4KDT, 4L4T, 4L4V, 4LCW, 4LHU, 4MNQ
標識
代號	B2M
擴展標識	遺傳學：109700 鼠基因：88127 同源基因：2987 ChEMBL: 1741302 GeneCards: B2M Gene
基因本體論描述
分子功能	· protein binding;
細胞成分	· Golgi membrane; · extracellular region; · extracellular space; · endoplasmic reticulum lumen; · plasma membrane; · external side of plasma membrane; · ER to Golgi transport vesicle membrane; · phagocytic vesicle membrane; · early endosome membrane; · early endosome lumen; · MHC class I protein complex;
生物過程	· positive regulation of T cell mediated cytotoxicity; · response to molecule of bacterial origin; · antigen processing and presentation of peptide antigen via MHC class I; · antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent; · antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent; · antigen processing and presentation of exogenous protein antigen via MHC class Ib, TAP-dependent; · viral reproduction; · cytokine-mediated signaling pathway; · T cell differentiation in thymus; · protein refolding; · response to drug; · antigen processing and presentation of exogenous peptide antigen via MHC class I; · innate immune response; · response to cadmium ion; · regulation of defense response to virus by virus; · regulation of immune response; · interferon-gamma-mediated signaling pathway;
	Sources: Amigo / QuickGO
RNA表達模式
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直系同源體
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A+醫學百科 >> Β2微球蛋白

β2微球蛋白

β2微球蛋白（英語：β₂ microglobulin，縮寫B2M）是第一類主要組織相容性抗原分子的組成部分之一，在所有有核的細胞中（即不包括紅細胞）都有表達^[1]^[2]。在人類基因組中，β2微球蛋白^[3]由B2M 基因所編碼^[2]^[4]。

結構功能

β2微球蛋白位于細胞表面的α₃鏈旁，但與α鏈不同的是，β₂并沒有跨膜區域，且為非共價結合。在β₂的“上方”（指遠離細胞膜的一端）為α鏈的α₁區和α₂區，構成了MHC的肽結合區。

β2微球蛋白不止參與形成第一類MHC分子，還能組成與之類似的表面受體，如CD1等。

β2微球蛋白還有一個功能，就是能與HFE (基因)結合，來調控鐵調素在肝臟中的表達，which targets the iron transporter 膜鐵轉運蛋白 on the cytoplasmic membrane of 腸上皮細胞和巨噬細胞 for degradation resulting in decreased iron uptake from food and iron release from recycled red blood cells respectively. 該功能的喪失會導致鐵過量和血色沉著病。

臨床意義

In patients on long-term hemodialysis, it can aggregate into amyloid fibers that deposit in joint spaces, a disease known as dialysis-related amyloidosis.

Mice models deficient for the β₂ microglobulin gene have been engineered. These mice demonstrate that β₂ microglobulin is necessary for cell surface expression of MHC class I and stability of the peptide binding groove. In fact, in the absence of β₂ microglobulin, very limited amounts of MHC class I (classical and non-classical) molecules can be detected on the surface. In the absence of MHC class I, CD8 T cells cannot develop. (CD8 T cells are a subset of T cells involved in the development of acquired immunity.) Low levels of β₂ microglobulin can indicate non-progression of HIV.

Levels of beta-2 microglobulin can be elevated in multiple myeloma and lymphoma, though in these cases primary amyloidosis (amyloid light chain) and secondary amyloidosis (amyloid associated protein) are more common. The normal value of beta-2 microglobulin is <2 mg/L.^[5] However, with respect to multiple myeloma, the levels of beta2-microglobulin may also be at the other end of the spectrum. Diagnostic testing for multiple myeloma includes obtaining the beta2-microglobulin level, for this level is an important prognostic indicator. A patient with a level <4 mg/L is expected to have a median survival of 43 months, while one with a level >4 mg/L has a median survival of only 12 months.^[6] Beta-2 microglobulin levels cannot, however, distinguish between monoclonal gammopathy of uncertain significance (MGUS), which has a better prognosis, and smouldering (low grade) myeloma.^[7]^[8]

參考文獻

↑ Entrez Gene: Beta-2-microglobulin.
↑ ^2.0 ^2.1 Güssow D, Rein R, Ginjaar I, Hochstenbach F, Seemann G, Kottman A, Ploegh HL. The human beta 2-microglobulin gene. Primary structure and definition of the transcriptional unit. J. Immunol.. 1 November 1987, 139 (9): 3132–8. PMID 3312414.
↑ Cunningham BA, Wang JL, Bergg?rd I, Peterson PA. The complete amino acid sequence of beta 2-microglobulin. Biochemistry. November 1973, 12 (24): 4811–22. doi:10.1021/bi00748a001. PMID 4586824.
↑ Suggs SV, Wallace RB, Hirose T, Kawashima EH, Itakura K. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC349099/ Use of synthetic oligonucleotides as hybridization probes: isolation of cloned cDNA sequences for human beta 2-microglobulin]. Proc. Natl. Acad. Sci. U.S.A.. November 1981, 78 (11): 6613–7. doi:10.1073/pnas.78.11.6613. PMID 6171820. PMC 349099.
↑ Pignone M, Nicoll D; McPhee SJ. Pocket guide to diagnostic tests. 4th. New York: McGraw-Hill. 2004: pp. 191. ISBN 0-07-141184-4.
↑ Munshi NC, Longo DL, Anderson KC. Chapter 111: Plasma Cell Disorders//Loscalzo J, Longo DL, Fauci AS, Dennis LK, Hauser SL. Harrison's Principles of Internal Medicine. 18th. McGraw-Hill Professional. 2011: pp. 936–44. ISBN 0-07-174889-X.
↑ Rajkumar S. V. "MGUS and Smoldering Multiple Myeloma: Update on Pathogenesis, Natural History, and Management." Hematology, American Society of Hematology Education Program. doi: 10.1182/asheducation-2005.1.340 (ASH Education Book January 1, 2005 vol. 2005 no. 1 340-345) Accessed 23 May 2014.
↑ Bataille R. and Klein B. "Serum levels of beta-2 microglobulin and interleukin-6 to differentiate monoclonal gammopathy of uncertain significance." Blood 1992 80(9) p2433 Accessed 23 May 2014.

延伸閱讀

Huang WC, Havel JJ, Zhau HE, Qian WP, Lue HW, Chu CY, Nomura T, Chung LW.. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC3032570/ β2-Microglobulin Signaling Blockade Inhibited Androgen Receptor Axis and Caused Apoptosis in Human Prostate Cancer Cells]. Clin. Cancer Res.. 2008, 14 (17): 5341–7. doi:10.1158/1078-0432.CCR-08-0793. PMID 18765525. PMC 3032570.
Huang WC, Wu D, Xie Z, Zhau HE, Nomura T, Zayzafoon M, Pohl J, Hsieh CL, Weitzmann MN, Farach-Carson MC, Chung LW.. Beta2-microglobulin is a signaling and growth-promoting factor for human prostate cancer bone metastasis. Cancer Res.. 2006, 66 (18): 9108–16. doi:10.1158/0008-5472.CAN-06-1996. PMID 16982753.
Winchester JF, Salsberg JA, Levin NW. Beta-2 microglobulin in ESRD: an in-depth review. Advances in renal replacement therapy. 2004, 10 (4): 279–309. doi:10.1053/j.arrt.2003.11.003. PMID 14681859.
Krangel MS, Orr HT, Strominger JL. Assembly and maturation of HLA-A and HLA-B antigens in vivo. Cell. 1980, 18 (4): 979–91. doi:10.1016/0092-8674(79)90210-1. PMID 93026.
Okon M, Bray P, Vuceli? D. 1H NMR assignments and secondary structure of human beta 2-microglobulin in solution. Biochemistry. 1992, 31 (37): 8906–15. doi:10.1021/bi00152a030. PMID 1390678.
Guo HC, Jardetzky TS, Garrett TP et al.. Different length peptides bind to HLA-Aw68 similarly at their ends but bulge out in the middle. Nature. 1992, 360 (6402): 364–6. doi:10.1038/360364a0. PMID 1448153.
Gattoni-Celli S, Kirsch K, Timpane R, Isselbacher KJ. Beta 2-microglobulin gene is mutated in a human colon cancer cell line (HCT) deficient in the expression of HLA class I antigens on the cell surface. Cancer Res.. 1992, 52 (5): 1201–4. PMID 1737380.
Saper MA, Bjorkman PJ, Wiley DC. Refined structure of the human histocompatibility antigen HLA-A2 at 2.6 A resolution. J. Mol. Biol.. 1991, 219 (2): 277–319. doi:10.1016/0022-2836(91)90567-P. PMID 2038058.
Caruana RJ, Lobel SA, Leffell MS et al.. Tumor necrosis factor, interleukin-1 and beta 2-microglobulin levels in chronic hemodialysis patients. The International journal of artificial organs. 1991, 13 (12): 794–8. PMID 2289831.
Connors LH, Shirahama T, Skinner M et al.. In vitro formation of amyloid fibrils from intact beta 2-microglobulin. Biochem. Biophys. Res. Commun.. 1985, 131 (3): 1063–8. doi:10.1016/0006-291X(85)90198-6. PMID 2413854.
Hochman JH, Shimizu Y, DeMars R, Edidin M. Specific associations of fluorescent beta-2-microglobulin with cell surfaces. The affinity of different H-2 and HLA antigens for beta-2-microglobulin. J. Immunol.. 1988, 140 (7): 2322–9. PMID 2450918.
Homma N, Gejyo F, Isemura M, Arakawa M. Collagen-binding affinity of beta-2-microglobulin, a preprotein of hemodialysis-associated amyloidosis. Nephron. 1989, 53 (1): 37–40. doi:10.1159/000185699. PMID 2674742.
Bataille R, Grenier J, Commes T. In vitro production of beta 2 microglobulin by human myeloma cells. Cancer Invest.. 1988, 6 (3): 271–7. doi:10.3109/07357908809080649. PMID 3048575.
H?nig R, Marsen T, Schad S et al.. Correlation of beta-2-microglobulin concentration changes to changes of distribution volume. The International journal of artificial organs. 1989, 11 (6): 459–64. PMID 3060434.
Bjorkman PJ, Saper MA, Samraoui B et al.. Structure of the human class I histocompatibility antigen, HLA-A2. Nature. 1987, 329 (6139): 506–12. doi:10.1038/329506a0. PMID 3309677.
Güssow D, Rein R, Ginjaar I et al.. The human beta 2-microglobulin gene. Primary structure and definition of the transcriptional unit. J. Immunol.. 1987, 139 (9): 3132–8. PMID 3312414.
Cunningham BA, Wang JL, Bergg?rd I, Peterson PA. The complete amino acid sequence of beta 2-microglobulin. Biochemistry. 1974, 12 (24): 4811–22. doi:10.1021/bi00748a001. PMID 4586824.
Suggs SV, Wallace RB, Hirose T et al.. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC349099/ Use of synthetic oligonucleotides as hybridization probes: isolation of cloned cDNA sequences for human beta 2-microglobulin]. Proc. Natl. Acad. Sci. U.S.A.. 1982, 78 (11): 6613–7. doi:10.1073/pnas.78.11.6613. PMID 6171820. PMC 349099.
Momoi T, Suzuki M, Titani K et al.. Amino acid sequence of a modified beta 2-microglobulin in renal failure patient urine and long-term dialysis patient blood. Clin. Chim. Acta. 1995, 236 (2): 135–44. doi:10.1016/0009-8981(95)06039-G. PMID 7554280.
Collins EJ, Garboczi DN, Karpusas MN, Wiley DC. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC42670/ The three-dimensional structure of a class I major histocompatibility complex molecule missing the alpha 3 domain of the heavy chain]. Proc. Natl. Acad. Sci. U.S.A.. 1995, 92 (4): 1218–21. doi:10.1073/pnas.92.4.1218. PMID 7862664. PMC 42670.
Matoba R, Okubo K, Hori N et al.. The addition of 5'-coding information to a 3'-directed cDNA library improves analysis of gene expression. Gene. 1994, 146 (2): 199–207. doi:10.1016/0378-1119(94)90293-3. PMID 8076819.
Wang Z, Cao Y, Albino AP et al.. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC288010/ Lack of HLA class I antigen expression by melanoma cells SK-MEL-33 caused by a reading frameshift in beta 2-microglobulin messenger RNA]. J. Clin. Invest.. 1993, 91 (2): 684–92. doi:10.1172/JCI116249. PMID 8432869. PMC 288010.

外部鏈接

MeSH（醫學主題詞）上面的beta+2-Microglobulin

參考來源

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Β2微球蛋白

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結構功能

臨床意義

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