Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients
Energy metabolism could influence amyotrophic lateral sclerosis (ALS) and progressive lateral sclerosis (PLS) pathogenesis and the response to therapy. We developed a novel assay to simultaneously assess mitochondrial content and membrane potential in patients' skin fibroblasts. In ALS and PLS...
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| Veröffentlicht in: | Annals of neurology 2014-10, Vol.76 (4), p.620-624 |
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| container_end_page | 624 |
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| container_issue | 4 |
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| container_title | Annals of neurology |
| container_volume | 76 |
| creator | Kirk, Kathryne Gennings, Chris Hupf, Jonathan C. Tadesse, Saba D'Aurelio, Marilena Kawamata, Hibiki Valsecchi, Federica Mitsumoto, Hiroshi Manfredi, Giovanni |
| description | Energy metabolism could influence amyotrophic lateral sclerosis (ALS) and progressive lateral sclerosis (PLS) pathogenesis and the response to therapy. We developed a novel assay to simultaneously assess mitochondrial content and membrane potential in patients' skin fibroblasts. In ALS and PLS fibroblasts, membrane potential was increased and mitochondrial content decreased, relative to healthy controls. In ALS higher mitochondrial membrane potential correlated with age at diagnosis, and in PLS it correlated with disease severity. These unprecedented findings in ALS and PLS fibroblasts could shed new light onto disease pathogenesis and help in developing biomarkers to predict disease evolution and the individual response to therapy in motor neuron diseases. Ann Neurol 2014;76:620–624 |
| doi_str_mv | 10.1002/ana.24244 |
| format | Article |
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We developed a novel assay to simultaneously assess mitochondrial content and membrane potential in patients' skin fibroblasts. In ALS and PLS fibroblasts, membrane potential was increased and mitochondrial content decreased, relative to healthy controls. In ALS higher mitochondrial membrane potential correlated with age at diagnosis, and in PLS it correlated with disease severity. These unprecedented findings in ALS and PLS fibroblasts could shed new light onto disease pathogenesis and help in developing biomarkers to predict disease evolution and the individual response to therapy in motor neuron diseases. 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We developed a novel assay to simultaneously assess mitochondrial content and membrane potential in patients' skin fibroblasts. In ALS and PLS fibroblasts, membrane potential was increased and mitochondrial content decreased, relative to healthy controls. In ALS higher mitochondrial membrane potential correlated with age at diagnosis, and in PLS it correlated with disease severity. These unprecedented findings in ALS and PLS fibroblasts could shed new light onto disease pathogenesis and help in developing biomarkers to predict disease evolution and the individual response to therapy in motor neuron diseases. Ann Neurol 2014;76:620–624</description><subject>Adult</subject><subject>Aged</subject><subject>Aldehydes</subject><subject>Amyotrophic lateral sclerosis</subject><subject>Amyotrophic Lateral Sclerosis - pathology</subject><subject>Biomarkers</subject><subject>Disease</subject><subject>Energy Metabolism - physiology</subject><subject>Fibroblasts - pathology</subject><subject>Humans</subject><subject>Male</subject><subject>Membrane Potential, Mitochondrial - physiology</subject><subject>Middle Aged</subject><subject>Motor Neuron Disease - pathology</subject><subject>Pathogenesis</subject><subject>Rhodamines - metabolism</subject><subject>Skin - pathology</subject><issn>0364-5134</issn><issn>1531-8249</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqN0T9v1DAYBnALgei1MPAFUCSWdkj7-n88XlsooOpYKjFaTmIXt7k4-M0BN_DdMb22AwKJxfbw8yO_fgh5ReGYArATN7pjJpgQT8iCSk7rhgnzlCyAK1FLysUe2Ue8AQCjKDwne0yCAdOwBfl5GpMffb72c-yqtcu3PmMVxwpvyxJim1M7OJyxSqHCKWXXF-fW2zTnNH0p58HNPruhwm7wOWHEyo19NeV0nT1i_Ob_IiY3Rz_O-II8C25A__J-PyBX795enb2vLz9dfDhbXtadZEbUVKqWM902PISm70UPwinNoNFtMME4cGCcE4EarnnLOqEa2TLtG9XTTgV-QA53seVVXzceZ7uO2PlhcKNPG7RUUaqY0pL_BwUjQXHDCn3zB71JmzyWOe4UaAApizraqa6MjtkHO-VY_nlrKdjf7dnSnr1rr9jX94mbdu37R_lQVwEnO_A9Dn777yS7XC0fIuvdjYiz__F4o_RsleZa2s-rC3t-3qwENB_tKf8FDO-z9A</recordid><startdate>201410</startdate><enddate>201410</enddate><creator>Kirk, Kathryne</creator><creator>Gennings, Chris</creator><creator>Hupf, Jonathan C.</creator><creator>Tadesse, Saba</creator><creator>D'Aurelio, Marilena</creator><creator>Kawamata, Hibiki</creator><creator>Valsecchi, Federica</creator><creator>Mitsumoto, Hiroshi</creator><creator>Manfredi, Giovanni</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</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>7TK</scope><scope>7U7</scope><scope>C1K</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>201410</creationdate><title>Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients</title><author>Kirk, Kathryne ; 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| ispartof | Annals of neurology, 2014-10, Vol.76 (4), p.620-624 |
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| language | eng |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Adult Aged Aldehydes Amyotrophic lateral sclerosis Amyotrophic Lateral Sclerosis - pathology Biomarkers Disease Energy Metabolism - physiology Fibroblasts - pathology Humans Male Membrane Potential, Mitochondrial - physiology Middle Aged Motor Neuron Disease - pathology Pathogenesis Rhodamines - metabolism Skin - pathology |
| title | Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients |
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