Skeletal muscle fiber function and rate of disease progression in amyotrophic lateral sclerosis

The contractile properties of single muscle fibers reflect the functional status of muscle at the cellular level and have not been described in amyotrophic lateral sclerosis (ALS). Chemically skinned single muscle fibers (n = 173), obtained by needle biopsy from six men with ALS, were activated with...

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Veröffentlicht in:Muscle & nerve 2002-11, Vol.26 (5), p.636-643
Hauptverfasser: Krivickas, Lisa S., Yang, Jung-In, Kim, Sang-Kyu, Frontera, Walter R.
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creator Krivickas, Lisa S.
Yang, Jung-In
Kim, Sang-Kyu
Frontera, Walter R.
description The contractile properties of single muscle fibers reflect the functional status of muscle at the cellular level and have not been described in amyotrophic lateral sclerosis (ALS). Chemically skinned single muscle fibers (n = 173), obtained by needle biopsy from six men with ALS, were activated with Ca2+, allowing maximal force measurements and specific force (SF) estimates. Maximum unloaded shortening velocity (Vo) was determined using the slack test. The results were compared with muscle from healthy controls. Markers of disease progression included rate of change of ALS functional rating scale score, rate of change of forced vital capacity, and disease duration. Compared with controls, ALS patients had decreased whole muscle SF (measured by a combination of computerized tomography and isokinetic testing) but normal single fiber SF. The Vo was greater for type I fibers in ALS. Patients with slower disease progression had increased single fiber size and a high percentage of hybrid fibers (expressing multiple myosin heavy chain isoforms). A needle biopsy obtained at the time of ALS diagnosis may assist with predicting rate of disease progression. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 636–643, 2002
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Chemically skinned single muscle fibers (n = 173), obtained by needle biopsy from six men with ALS, were activated with Ca2+, allowing maximal force measurements and specific force (SF) estimates. Maximum unloaded shortening velocity (Vo) was determined using the slack test. The results were compared with muscle from healthy controls. Markers of disease progression included rate of change of ALS functional rating scale score, rate of change of forced vital capacity, and disease duration. Compared with controls, ALS patients had decreased whole muscle SF (measured by a combination of computerized tomography and isokinetic testing) but normal single fiber SF. The Vo was greater for type I fibers in ALS. Patients with slower disease progression had increased single fiber size and a high percentage of hybrid fibers (expressing multiple myosin heavy chain isoforms). A needle biopsy obtained at the time of ALS diagnosis may assist with predicting rate of disease progression. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 636–643, 2002</description><subject>Adult</subject><subject>Age Factors</subject><subject>Aged</subject><subject>Amyotrophic Lateral Sclerosis - pathology</subject><subject>Amyotrophic Lateral Sclerosis - physiopathology</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>contractile properties</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Disease Progression</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>motor neuron disease</subject><subject>muscle biopsy</subject><subject>Muscle Contraction - physiology</subject><subject>Muscle Fibers, Skeletal - pathology</subject><subject>Muscle Weakness - pathology</subject><subject>Muscle Weakness - physiopathology</subject><subject>Muscle, Skeletal - pathology</subject><subject>Muscle, Skeletal - physiopathology</subject><subject>myosin heavy chain</subject><subject>Neurology</subject><subject>shortening velocity</subject><subject>single fiber</subject><subject>specific force</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2002</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kM1u1DAUhS0EokNhwQsgb0DqItQ_iX-WqIIBaQCJUsrOcuxrMHWSwU5U5u3xMANdsfKV_N3vHh2EnlLykhLCzoel1IF18h5aUaJl03Za3UcrQlvVCK6_nqBHpfwghFAl5EN0QllLGFPdCpnLG0gw24SrxCXAIfaQcVhGN8dpxHb0ONsZ8BSwjwVsAbzN07cMpez_Y0WG3TTnafs9Opwqmqtsr8pTieUxehBsKvDk-J6iqzevP1-8bTYf1-8uXm0ax1UNHLzkTCgBVAjXUrCity0nvRROBU075UhohQughVatCtIT4TwV3Evqqe_5KXpx8NZwPxcosxlicZCSHWFaipFMcKY7UsGzA-hqvpIhmG2Og807Q4nZt2lqEeZPm5V9dpQu_QD-jjzWV4HnR8AWZ1PIdnSx3HFca8Ulq9z5gbuNCXb_v2jeX13-Pd0cNmKZ4de_DZtvjJBcdub6w7pOm_bL-pqbT_w3jW2cFw</recordid><startdate>200211</startdate><enddate>200211</enddate><creator>Krivickas, Lisa S.</creator><creator>Yang, Jung-In</creator><creator>Kim, Sang-Kyu</creator><creator>Frontera, Walter R.</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>200211</creationdate><title>Skeletal muscle fiber function and rate of disease progression in amyotrophic lateral sclerosis</title><author>Krivickas, Lisa S. ; Yang, Jung-In ; Kim, Sang-Kyu ; Frontera, Walter R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3897-fd732686e166c41ea6ba430b76c8f9158c0f46cfe969848f7d06cd163d71d1db3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2002</creationdate><topic>Adult</topic><topic>Age Factors</topic><topic>Aged</topic><topic>Amyotrophic Lateral Sclerosis - pathology</topic><topic>Amyotrophic Lateral Sclerosis - physiopathology</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>contractile properties</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Disease Progression</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>motor neuron disease</topic><topic>muscle biopsy</topic><topic>Muscle Contraction - physiology</topic><topic>Muscle Fibers, Skeletal - pathology</topic><topic>Muscle Weakness - pathology</topic><topic>Muscle Weakness - physiopathology</topic><topic>Muscle, Skeletal - pathology</topic><topic>Muscle, Skeletal - physiopathology</topic><topic>myosin heavy chain</topic><topic>Neurology</topic><topic>shortening velocity</topic><topic>single fiber</topic><topic>specific force</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Krivickas, Lisa S.</creatorcontrib><creatorcontrib>Yang, Jung-In</creatorcontrib><creatorcontrib>Kim, Sang-Kyu</creatorcontrib><creatorcontrib>Frontera, Walter R.</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle &amp; nerve</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Krivickas, Lisa S.</au><au>Yang, Jung-In</au><au>Kim, Sang-Kyu</au><au>Frontera, Walter R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Skeletal muscle fiber function and rate of disease progression in amyotrophic lateral sclerosis</atitle><jtitle>Muscle &amp; nerve</jtitle><addtitle>Muscle Nerve</addtitle><date>2002-11</date><risdate>2002</risdate><volume>26</volume><issue>5</issue><spage>636</spage><epage>643</epage><pages>636-643</pages><issn>0148-639X</issn><eissn>1097-4598</eissn><coden>MUNEDE</coden><abstract>The contractile properties of single muscle fibers reflect the functional status of muscle at the cellular level and have not been described in amyotrophic lateral sclerosis (ALS). Chemically skinned single muscle fibers (n = 173), obtained by needle biopsy from six men with ALS, were activated with Ca2+, allowing maximal force measurements and specific force (SF) estimates. Maximum unloaded shortening velocity (Vo) was determined using the slack test. The results were compared with muscle from healthy controls. Markers of disease progression included rate of change of ALS functional rating scale score, rate of change of forced vital capacity, and disease duration. Compared with controls, ALS patients had decreased whole muscle SF (measured by a combination of computerized tomography and isokinetic testing) but normal single fiber SF. The Vo was greater for type I fibers in ALS. Patients with slower disease progression had increased single fiber size and a high percentage of hybrid fibers (expressing multiple myosin heavy chain isoforms). 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subjects Adult
Age Factors
Aged
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - physiopathology
Biological and medical sciences
Biopsy
contractile properties
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Disease Progression
Humans
Male
Medical sciences
Middle Aged
motor neuron disease
muscle biopsy
Muscle Contraction - physiology
Muscle Fibers, Skeletal - pathology
Muscle Weakness - pathology
Muscle Weakness - physiopathology
Muscle, Skeletal - pathology
Muscle, Skeletal - physiopathology
myosin heavy chain
Neurology
shortening velocity
single fiber
specific force
title Skeletal muscle fiber function and rate of disease progression in amyotrophic lateral sclerosis
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