14.6.1 POJA-L6325 Nemaline myopathy III, ultrastructure of nemaline rods in skeletal muscle (human)
14.6.1 POJA-L6325 Nemaline myopathy III, ultrastructure of nemaline rods in skeletal muscle (human)
(By courtesy of L. Eshuis BSc Section Electron microscopy, Department of Pathology, Radboud university medical center, Nijmegen, The Netherlands)
Title: Nemaline myopathy III, ultrastructure of nemaline rods in skeletal muscle (human)
Description:
(1): High magnification of rod-like structures of Z-line material due to mutations in predominantly actin and nebulin genes.
(2): Z-lines. At the right side above the top (2) the begin of the disruption of a sarcomere. At the left several short pieces of sectioned rods, there is a loss of the A- and I-bands. In this area disruption and disorganisation of the normal contractile network occur.
For review see:
Keywords/Mesh: locomotor system, skeletal muscle, striated muscle, neuromuscular disease, congenital myopathy, nemaline myopathy, rod body myopathy, nemaline body, rod, Z-line, a-actinin, electron microscopy, pathology, POJA collection
Title: Nemaline myopathy III, ultrastructure of nemaline rods in skeletal muscle (human)
Description:
(1): High magnification of rod-like structures of Z-line material due to mutations in predominantly actin and nebulin genes.
(2): Z-lines. At the right side above the top (2) the begin of the disruption of a sarcomere. At the left several short pieces of sectioned rods, there is a loss of the A- and I-bands. In this area disruption and disorganisation of the normal contractile network occur.
For review see:
- Nemaline myopathies: a current view Caroline A Sewry 1 2, Jenni M Laitila , Carina Wallgren-Pettersson (J Muscle Res Cell Motil. 2019 Jun;40(2):111-126. doi: 10.1007/s10974-019-09519-9 ) https://pubmed.ncbi.nlm.nih.gov/31228046/
Keywords/Mesh: locomotor system, skeletal muscle, striated muscle, neuromuscular disease, congenital myopathy, nemaline myopathy, rod body myopathy, nemaline body, rod, Z-line, a-actinin, electron microscopy, pathology, POJA collection