PMID- 12832540
OWN - NLM
STAT- MEDLINE
DCOM- 20030805
LR  - 20200225
IS  - 1529-2401 (Electronic)
IS  - 0270-6474 (Print)
IS  - 0270-6474 (Linking)
VI  - 23
IP  - 12
DP  - 2003 Jun 15
TI  - Aberrant development of motor axons and neuromuscular synapses in MyoD-null mice.
PG  - 5161-9
AB  - Myogenic regulatory factors (MRFs), muscle-specific transcription factors, are 
      implicated in the activity-dependent regulation of nicotinic acetylcholine 
      receptor (AChR) subunit genes. Here we show, with immunohistochemistry, Western 
      blotting, and electron microscopy that MyoD, a member of the MRF family, also 
      plays a role in fetal synapse formation. In the diaphragm of 14.5 d gestation 
      (E14.5) wild-type and MyoD-/- mice, AChR clusters (the formation of which is 
      under a muscle intrinsic program) are confined to a centrally located endplate 
      zone. This distribution persists in wild-type adult muscles. However, beginning 
      at E15.5 and extending to the adult, innervated AChR clusters are distributed all 
      over the diaphragm of MyoD-/- mice, extending as far as the insertion of the 
      diaphragm into the ribs. In wild-type muscle, motor axons terminate on clusters 
      adjacent to the main intramuscular nerve; in MyoD-/- muscle, axonal bundles form 
      extensive secondary branches that terminate on the widely distributed clusters. 
      The number of AChR clusters on adult MyoD-/- and wild-type diaphram muscles is 
      similar. Junctional fold density is reduced at MyoD-/- endplates, and the 
      transition from the fetal (alpha, beta, gamma, delta) to adult-type (alpha, beta, 
      delta, epsilon) AChRs is markedly delayed. However, MyoD-/- mice assemble a 
      complex postsynaptic apparatus that includes muscle-specific kinase (MuSK), 
      rapsyn, erbB, and utrophin.
FAU - Wang, Zuo-Zhong
AU  - Wang ZZ
AD  - Department of Neurobiology, University of Pittsburgh School of Medicine, 
      Pittsburgh, Pennsylvania 15261, USA.
FAU - Washabaugh, Charles H
AU  - Washabaugh CH
FAU - Yao, Yun
AU  - Yao Y
FAU - Wang, Jun-Mei
AU  - Wang JM
FAU - Zhang, Lili
AU  - Zhang L
FAU - Ontell, Martin P
AU  - Ontell MP
FAU - Watkins, Simon C
AU  - Watkins SC
FAU - Rudnicki, Michael A
AU  - Rudnicki MA
FAU - Ontell, Marcia
AU  - Ontell M
LA  - eng
GR  - R01 AR046479/AR/NIAMS NIH HHS/United States
GR  - R01 NS038301/NS/NINDS NIH HHS/United States
GR  - AR-46479/AR/NIAMS NIH HHS/United States
GR  - NS-38301/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - J Neurosci
JT  - The Journal of neuroscience : the official journal of the Society for 
      Neuroscience
JID - 8102140
RN  - 0 (Agrin)
RN  - 0 (Muscle Proteins)
RN  - 0 (MyoD Protein)
RN  - 0 (Protein Subunits)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (peripheral membrane protein 43K)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Age Factors
MH  - Agrin/pharmacology
MH  - Animals
MH  - Axons/*pathology/ultrastructure
MH  - Cells, Cultured
MH  - Diaphragm/embryology/innervation/metabolism/pathology
MH  - Intercostal Muscles/innervation/metabolism/pathology
MH  - Mice
MH  - Mice, Inbred BALB C
MH  - Mice, Knockout
MH  - Motor Neurons/*pathology
MH  - Muscle Fibers, Skeletal/cytology/drug effects/metabolism
MH  - Muscle Proteins/metabolism
MH  - MyoD Protein/*genetics
MH  - Myoblasts/cytology
MH  - Nervous System Malformations/*genetics/pathology
MH  - Neuromuscular Junction/*pathology/ultrastructure
MH  - Protein Subunits/metabolism
MH  - Receptor Aggregation
MH  - Receptor Protein-Tyrosine Kinases/metabolism
MH  - Receptors, Cholinergic/metabolism
PMC - PMC6741161
EDAT- 2003/07/02 05:00
MHDA- 2003/08/06 05:00
PMCR- 2003/12/15
CRDT- 2003/07/02 05:00
PHST- 2003/07/02 05:00 [pubmed]
PHST- 2003/08/06 05:00 [medline]
PHST- 2003/07/02 05:00 [entrez]
PHST- 2003/12/15 00:00 [pmc-release]
AID - 23/12/5161 [pii]
AID - 10.1523/JNEUROSCI.23-12-05161.2003 [doi]
PST - ppublish
SO  - J Neurosci. 2003 Jun 15;23(12):5161-9. doi: 10.1523/JNEUROSCI.23-12-05161.2003.