Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1

Jiming Kong; Zuoshang Xu

doi:10.1523/JNEUROSCI.18-09-03241.1998

Article Information

DOI

https://doi.org/10.1523/JNEUROSCI.18-09-03241.1998

PubMed

9547233

Published By

Society for Neuroscience

History

Received November 7, 1997
Revision received February 12, 1998
Accepted February 18, 1998
First published May 1, 1998.

Version of record published May 1, 1998.

Author Information

Jiming Kong1 and
Zuoshang Xu2

¹Departments of Pharmacology and Molecular Toxicology and
²Cell Biology, University of Massachusetts Medical School, Worcester Foundation Campus, Shrewsbury, Massachusetts 01545

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Author contributions

Disclosures

Received November 7, 1997.
Revision received February 12, 1998.
Accepted February 18, 1998.

This work is supported by grants from National Institutes of Health (RO1NS35750-01), the ALS Association, and the Markey Charitable Trust. We thank Vivian Tung for technical assistance and Alonzo Ross and Diane Casey for critically reading this manuscript.
Correspondence should be addressed to Zuoshang Xu, Worcester Foundation, 222 Maple Avenue, Shrewsbury, MA 01545.

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	Abstract	Full	Pdf
Jan 1999	16	30	10
Feb 1999	15	24	5
Mar 1999	12	25	9
Dec 1999	2	12	13
Total 1999	45	91	37
Jan 2000	6	35	18
Feb 2000	11	59	21
Mar 2000	8	34	35
Apr 2000	7	31	21
May 2000	3	27	20
Jun 2000	4	13	13
Jul 2000	24	18	19
Aug 2000	8	30	29
Sep 2000	8	32	20
Oct 2000	4	42	26
Nov 2000	7	65	38
Dec 2000	3	18	13
Total 2000	93	404	273
Jan 2001	5	15	11
Feb 2001	4	27	19
Mar 2001	2	35	14
Apr 2001	4	37	25
May 2001	10	49	33
Jun 2001	1	26	19
Jul 2001	8	26	20
Aug 2001	4	17	15
Sep 2001	3	26	27
Oct 2001	4	23	15
Nov 2001	5	44	22
Dec 2001	5	26	20
Total 2001	55	351	240
Jan 2002	4	38	34
Feb 2002	7	27	14
Mar 2002	10	36	20
Apr 2002	5	41	37
May 2002	4	18	23
Jun 2002	8	20	15
Jul 2002	5	21	35
Aug 2002	3	23	26
Sep 2002	7	29	18
Oct 2002	10	40	39
Nov 2002	4	23	23
Dec 2002	5	22	23
Total 2002	72	338	307
Jan 2003	12	21	40
Feb 2003	12	30	30
Mar 2003	6	29	27
Apr 2003	5	23	24
May 2003	6	35	37
Jun 2003	2	13	19
Jul 2003	14	20	12
Aug 2003	11	27	17
Sep 2003	11	27	21
Oct 2003	13	26	30
Nov 2003	17	56	27
Dec 2003	21	52	33
Total 2003	130	359	317
Jan 2004	17	39	22
Feb 2004	12	37	28
Mar 2004	19	57	37
Apr 2004	18	40	24
May 2004	19	51	26
Jun 2004	14	40	24
Jul 2004	14	26	22
Aug 2004	25	39	32
Sep 2004	24	29	32
Oct 2004	12	52	36
Nov 2004	20	38	33
Dec 2004	17	30	25
Total 2004	211	478	341
Jan 2005	17	44	41
Feb 2005	20	73	37
Mar 2005	29	58	28
Apr 2005	13	47	32
May 2005	19	51	32
Jun 2005	8	34	40
Jul 2005	11	21	19
Aug 2005	15	28	26
Sep 2005	8	20	16
Oct 2005	12	26	22
Nov 2005	15	48	33
Dec 2005	10	30	18
Total 2005	177	480	344
Jan 2006	10	36	28
Feb 2006	10	45	23
Mar 2006	23	43	29
Apr 2006	12	34	29
May 2006	6	24	23
Jun 2006	8	13	8
Jul 2006	2	16	19
Aug 2006	7	24	18
Sep 2006	26	55	22
Oct 2006	16	48	23
Nov 2006	42	43	39
Dec 2006	42	50	32
Total 2006	204	431	293
Jan 2007	32	54	30
Feb 2007	28	43	26
Mar 2007	35	49	44
Apr 2007	29	26	31
May 2007	26	39	30
Jun 2007	19	24	18
Jul 2007	9	24	14
Aug 2007	14	25	13
Sep 2007	17	23	20
Oct 2007	20	31	25
Nov 2007	26	36	28
Dec 2007	32	19	19
Total 2007	287	393	298
Jan 2008	54	23	23
Feb 2008	35	39	18
Mar 2008	26	29	35
Apr 2008	30	26	30
May 2008	16	22	20
Jun 2008	11	30	25
Jul 2008	18	22	9
Aug 2008	14	23	22
Sep 2008	33	5	20
Oct 2008	34	17	24
Nov 2008	42	19	27
Dec 2008	15	13	18
Total 2008	328	268	271
Jan 2009	19	26	26
Feb 2009	26	23	26
Mar 2009	25	33	33
Apr 2009	21	19	33
May 2009	18	20	27
Jun 2009	16	21	15
Jul 2009	19	21	30
Aug 2009	12	17	22
Sep 2009	17	32	23
Oct 2009	32	21	34
Nov 2009	23	16	19
Dec 2009	28	21	20
Total 2009	256	270	308
Jan 2010	10	20	11
Feb 2010	14	22	17
Mar 2010	59	30	15
Apr 2010	42	24	26
May 2010	35	45	40
Jun 2010	19	31	20
Jul 2010	32	25	25
Aug 2010	14	17	20
Sep 2010	27	23	28
Oct 2010	53	61	40
Nov 2010	24	32	18
Dec 2010	13	19	16
Total 2010	342	349	276
Jan 2011	17	20	17
Feb 2011	29	32	19
Mar 2011	29	36	20
Apr 2011	46	33	24
May 2011	52	36	30
Jun 2011	22	27	28
Jul 2011	35	24	32
Aug 2011	26	23	19
Sep 2011	55	39	34
Oct 2011	43	33	29
Nov 2011	43	37	44
Dec 2011	37	33	21
Total 2011	434	373	317
Jan 2012	39	30	34
Feb 2012	39	29	31
Mar 2012	65	46	59
Apr 2012	28	30	23
May 2012	37	23	24
Jun 2012	38	16	18
Jul 2012	21	19	23
Aug 2012	32	19	13
Sep 2012	25	18	23
Oct 2012	43	17	29
Nov 2012	41	26	26
Dec 2012	52	17	13
Total 2012	460	290	316
Jan 2013	34	21	33
Feb 2013	37	14	18
Mar 2013	37	19	15
Apr 2013	32	26	42
May 2013	44	20	35
Jun 2013	19	15	24
Jul 2013	16	17	27
Aug 2013	18	9	20
Sep 2013	21	15	25
Oct 2013	33	27	31
Nov 2013	41	23	41
Dec 2013	22	15	30
Total 2013	354	221	341
Jan 2014	10	14	32
Feb 2014	15	27	34
Mar 2014	38	23	43
Apr 2014	30	23	19
May 2014	22	21	31
Jun 2014	33	20	26
Jul 2014	30	22	28
Aug 2014	35	17	35
Sep 2014	50	35	43
Oct 2014	39	10	31
Nov 2014	43	34	32
Dec 2014	35	22	19
Total 2014	380	268	373
Jan 2015	30	25	25
Feb 2015	30	15	28
Mar 2015	44	29	32
Apr 2015	34	26	20
May 2015	47	23	40
Jun 2015	24	19	17
Jul 2015	26	26	27
Aug 2015	15	15	9
Sep 2015	49	14	17
Oct 2015	25	29	32
Nov 2015	32	20	37
Dec 2015	31	26	13
Total 2015	387	267	297
Jan 2016	79	13	20
Feb 2016	28	20	21
Mar 2016	36	37	41
Apr 2016	32	30	28
May 2016	27	22	11
Jun 2016	29	19	18
Jul 2016	24	21	16
Aug 2016	27	24	14
Sep 2016	34	36	19
Oct 2016	23	27	13
Nov 2016	24	21	27
Dec 2016	19	22	28
Total 2016	382	292	256
Jan 2017	32	25	11
Feb 2017	10	30	14
Mar 2017	19	40	26
Apr 2017	14	18	8
May 2017	24	68	33
Jun 2017	11	16	18
Jul 2017	11	36	15
Aug 2017	13	17	10
Sep 2017	15	14	11
Oct 2017	48	59	24
Nov 2017	19	34	21
Dec 2017	7	24	11
Total 2017	223	381	202
Jan 2018	10	22	8
Feb 2018	11	20	11
Mar 2018	47	35	45
Apr 2018	8	41	37
May 2018	16	31	25
Jun 2018	13	25	16
Jul 2018	10	27	12
Aug 2018	13	22	19
Sep 2018	6	39	18
Oct 2018	27	40	27
Nov 2018	18	56	32
Dec 2018	7	22	14
Total 2018	186	380	264
Jan 2019	8	22	11
Feb 2019	10	25	24
Mar 2019	24	42	29
Apr 2019	18	47	36
May 2019	12	36	21
Jun 2019	4	26	12
Jul 2019	6	19	13
Aug 2019	7	39	31
Sep 2019	5	21	22
Oct 2019	16	50	22
Nov 2019	11	34	23
Dec 2019	4	28	18
Total 2019	125	389	262
Jan 2020	3	33	44
Feb 2020	6	20	12
Mar 2020	5	9	6
Apr 2020	9	33	22
May 2020	19	24	17
Jun 2020	5	13	7
Jul 2020	5	51	8
Aug 2020	5	24	7
Sep 2020	6	34	13
Oct 2020	13	40	16
Nov 2020	9	43	11
Dec 2020	15	54	11
Total 2020	100	378	174
Jan 2021	3	46	14
Feb 2021	11	35	18
Mar 2021	0	0	3
Total 2021	14	81	35
Total	5245	7532	6142