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Prevention of muscle fibrosis and myonecrosis in mdx mice by suramin, a TGF-β1 blocker

Fibrosis is a pathological feature observed in patients with Duchenne muscular dystrophy (DMD) and in mdx mice, the experimental model of DMD. We evaluated the effect of suramin, a transforming growth factor‐beta 1 (TGF‐β1) blocker, on fibrosis in mdx mice. mdx mice (6 months old) received suramin f...

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Published in:	Muscle & nerve 2011-01, Vol.43 (1), p.82-87
Main Authors:	Taniguti, Ana Paula Tiemi, Pertille, Adriana, Matsumura, Cíntia Yuri, Neto, Humberto Santo, Marques, Maria Julia
Format:	Article
Language:	English
Subjects:	Animals Biological and medical sciences Creatine Kinase - metabolism Disease Models, Animal Diseases of striated muscles. Neuromuscular diseases dystrophy Female Fibrosis Fundamental and applied biological sciences. Psychology Male mdx Medical sciences Mice Mice, Inbred C57BL Mice, Inbred mdx Muscle Fibers, Skeletal - pathology muscle fibrosis Muscle, Skeletal - pathology Necrosis Neurology Physical Conditioning, Animal - adverse effects Striated muscle. Tendons suramin Suramin - pharmacology Suramin - therapeutic use TGF-β1 Transforming Growth Factor beta1 - antagonists & inhibitors Transforming Growth Factor beta1 - physiology Vertebrates: osteoarticular system, musculoskeletal system
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creator	Taniguti, Ana Paula Tiemi Pertille, Adriana Matsumura, Cíntia Yuri Neto, Humberto Santo Marques, Maria Julia
description	Fibrosis is a pathological feature observed in patients with Duchenne muscular dystrophy (DMD) and in mdx mice, the experimental model of DMD. We evaluated the effect of suramin, a transforming growth factor‐beta 1 (TGF‐β1) blocker, on fibrosis in mdx mice. mdx mice (6 months old) received suramin for 7 weeks. Suramin‐ and saline‐treated (control) mdx mice performed exercise on a treadmill to worsen disease progression. Immunoblotting showed an increase of TGF‐β1 in mdx diaphragm, limb, and cardiac muscles. Suramin decreased creatine kinase in mdx mice and attenuated fibrosis in all muscles studied, except for cardiac muscle. Suramin protected limb muscles against damage and reduced the exercise‐induced loss of strength over time. These findings support a role for TGF‐β1 in fibrinogenesis and myonecrosis during the later stages of disease in mdx mice. Suramin might be a useful therapeutic alternative for the treatment of dystrophinopathies. Muscle Nerve, 2011
doi_str_mv	10.1002/mus.21869
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Tendons</subject><subject>suramin</subject><subject>Suramin - pharmacology</subject><subject>Suramin - therapeutic use</subject><subject>TGF-β1</subject><subject>Transforming Growth Factor beta1 - antagonists & inhibitors</subject><subject>Transforming Growth Factor beta1 - physiology</subject><subject>Vertebrates: osteoarticular system, musculoskeletal system</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><recordid>eNp10N1KHDEUB_BQLHXVXvgCJTelCB3NSeYjuRSpq2VbhSrrXcgkJ5A6H5rstO5r9UH6TB2d1V55FQK_8z-cPyH7wA6BMX7UDumQgyzVGzIDpqosL5TcIjMGucxKoW62yU5KPxljI6rekW0OwCTP8xlZXkb8hd0q9B3tPR2TbIPUhzr2KSRqOkfbdd-hnf6ho617oG2wSOs1TUM0beg-U0Ov5qfZ3z9A66a3txj3yFtvmoTvN-8uuT79cnVyli0u5ucnx4vMCiZUxq0tXY5lXXLnrAA0ALUH41DVPPe1NMxV6KXIDXghuPVlia7gyiCXHgqxSz5NuXexvx8wrXQbksWmMR32Q9KSs0pKpdgoDyb5eEqK6PVdDK2Jaw1MP9aox-P1U42j_bBJHeoW3Yt87m0EHzfAJGsaH01nQ_rvhAQJXIzuaHK_Q4Pr1zfqb9c_nldn00RIK3x4mTDxVpeVqAq9_D7XjF8ulmdfCz0X_wBhHpmH</recordid><startdate>201101</startdate><enddate>201101</enddate><creator>Taniguti, Ana Paula Tiemi</creator><creator>Pertille, Adriana</creator><creator>Matsumura, Cíntia Yuri</creator><creator>Neto, Humberto Santo</creator><creator>Marques, Maria Julia</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>201101</creationdate><title>Prevention of muscle fibrosis and myonecrosis in mdx mice by suramin, a TGF-β1 blocker</title><author>Taniguti, Ana Paula Tiemi ; Pertille, Adriana ; Matsumura, Cíntia Yuri ; Neto, Humberto Santo ; Marques, Maria Julia</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3039-2cc6d4e6b62ddc31ea11bf1ade9b24fb8a0d7ef834a1f332cf66ed529ae28f153</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>Creatine Kinase - metabolism</topic><topic>Disease Models, Animal</topic><topic>Diseases of striated muscles. 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subjects	Animals Biological and medical sciences Creatine Kinase - metabolism Disease Models, Animal Diseases of striated muscles. Neuromuscular diseases dystrophy Female Fibrosis Fundamental and applied biological sciences. Psychology Male mdx Medical sciences Mice Mice, Inbred C57BL Mice, Inbred mdx Muscle Fibers, Skeletal - pathology muscle fibrosis Muscle, Skeletal - pathology Necrosis Neurology Physical Conditioning, Animal - adverse effects Striated muscle. Tendons suramin Suramin - pharmacology Suramin - therapeutic use TGF-β1 Transforming Growth Factor beta1 - antagonists & inhibitors Transforming Growth Factor beta1 - physiology Vertebrates: osteoarticular system, musculoskeletal system
title	Prevention of muscle fibrosis and myonecrosis in mdx mice by suramin, a TGF-β1 blocker
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