MARC details
| 000 -LEADER |
|---|
| fixed length control field |
03190nam a22005777a 4500 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION |
|---|
| fixed length control field |
150313s20142014 xxu||||| |||| 00| 0 eng d |
| 040 ## - CATALOGING SOURCE |
|---|
| Original cataloging agency |
Ovid MEDLINE(R) |
| 099 ## - LOCAL FREE-TEXT CALL NUMBER (OCLC) |
|---|
| PMID |
24967968 |
| 245 ## - TITLE STATEMENT |
|---|
| Title |
Dysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion. |
| 251 ## - Source |
|---|
| Source |
Cell Death & Disease. 5:e1306, 2014. |
| 252 ## - Abbreviated Source |
|---|
| Abbreviated source |
Cell Death Dis. 5:e1306, 2014. |
| 253 ## - Journal Name |
|---|
| Journal name |
Cell death & disease |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
|---|
| Year |
2014 |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
|---|
| Manufacturer |
FY2015 |
| 266 ## - Date added to catalog |
|---|
| Date added to catalog |
2015-03-17 |
| 520 ## - SUMMARY, ETC. |
|---|
| Abstract |
Dysferlin deficiency compromises the repair of injured muscle, but the underlying cellular mechanism remains elusive. To study this phenomenon, we have developed mouse and human myoblast models for dysferlinopathy. These dysferlinopathic myoblasts undergo normal differentiation but have a deficit in their ability to repair focal injury to their cell membrane. Imaging cells undergoing repair showed that dysferlin-deficit decreased the number of lysosomes present at the cell membrane, resulting in a delay and reduction in injury-triggered lysosomal exocytosis. We find repair of injured cells does not involve formation of intracellular membrane patch through lysosome-lysosome fusion; instead, individual lysosomes fuse with the injured cell membrane, releasing acid sphingomyelinase (ASM). ASM secretion was reduced in injured dysferlinopathic cells, and acute treatment with sphingomyelinase restored the repair ability of dysferlinopathic myoblasts and myofibers. Our results provide the mechanism for dysferlin-mediated repair of skeletal muscle sarcolemma and identify ASM as a potential therapy for dysferlinopathy. |
| 546 ## - LANGUAGE NOTE |
|---|
| Language note |
English |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
*Membrane Proteins/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
*Muscle Proteins/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
*Myoblasts, Skeletal/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
*Sarcolemma/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
*Sphingomyelin Phosphodiesterase/se [Secretion] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Animals |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Cell Line |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Distal Myopathies/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Distal Myopathies/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Distal Myopathies/pa [Pathology] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Distal Myopathies/th [Therapy] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Exocytosis |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Humans |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Membrane Proteins/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Mice |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Muscle Proteins/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Muscular Atrophy/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Muscular Atrophy/me [Metabolism] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Muscular Atrophy/pa [Pathology] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Muscular Atrophy/th [Therapy] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Myoblasts, Skeletal/pa [Pathology] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Sarcolemma/ge [Genetics] |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
|---|
| Topical term or geographic name entry element |
Sarcolemma/pa [Pathology] |
| 651 ## - SUBJECT ADDED ENTRY--GEOGRAPHIC NAME |
|---|
| Institution |
MedStar Washington Hospital Center |
| 656 ## - INDEX TERM--OCCUPATION |
|---|
| Department |
Emergency Medicine |
| 657 ## - INDEX TERM--FUNCTION |
|---|
| Medline publication type |
Journal Article |
| 657 ## - INDEX TERM--FUNCTION |
|---|
| Medline publication type |
Research Support, N.I.H., Extramural |
| 657 ## - INDEX TERM--FUNCTION |
|---|
| Medline publication type |
Research Support, Non-U.S. Gov't |
| 657 ## - INDEX TERM--FUNCTION |
|---|
| Medline publication type |
Research Support, U.S. Gov't, Non-P.H.S. |
| 700 ## - ADDED ENTRY--PERSONAL NAME |
|---|
| Local Authors |
Wilson, Matthew D |
| 790 ## - Authors |
|---|
| All authors |
Bashir R, Bhat R, Bigot A, Defour A, Jaiswal JK, Nagaraju K, Van der Meulen JH |
| 856 ## - ELECTRONIC LOCATION AND ACCESS |
|---|
| DOI |
<a href="http://dx.doi.org/10.1038/cddis.2014.272">http://dx.doi.org/10.1038/cddis.2014.272</a> |
| Public note |
http://dx.doi.org/10.1038/cddis.2014.272 |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) |
|---|
| Koha item type |
Journal Article |
| Item type description |
Article |
