{"id":994,"date":"2026-04-11T22:28:01","date_gmt":"2026-04-11T22:28:01","guid":{"rendered":"http:\/\/wmtc2006.com\/?p=994"},"modified":"2026-04-11T22:28:01","modified_gmt":"2026-04-11T22:28:01","slug":"on-atpase-staining-there-was-type-i-fiber-predominance-and-hypertrophy-most-type-ii-fibers-were-atrophic","status":"publish","type":"post","link":"https:\/\/wmtc2006.com\/?p=994","title":{"rendered":"\ufeffOn ATPase staining, there was type I fiber predominance and hypertrophy; most type II fibers were atrophic"},"content":{"rendered":"<p>\ufeffOn ATPase staining, there was type I fiber predominance and hypertrophy; most type II fibers were atrophic. rod-shaped inclusions or nemaline bodies in muscle fibers14. Several clinical types of NEM are recognized based on the age of disease onset and severity of muscle weakness, ranging from a severe neonatal often lethal subtype to milder non-progressive or slowly progressive forms that present in infancy, childhood or adulthood57. Muscle weakness may be diffuse, but it is usually often most pronounced in the face, neck and proximal limb muscles. Pharyngeal and respiratory muscles are frequently affected5. Distal weakness appears late in the course of illness, but in some forms manifests at presentation8. The pattern of inheritance is also variable; autosomal recessive, autosomal dominant, and sporadic cases have been reported811. Nemaline bodies are rod-shaped structures continuous with and derived from the Z-disks12. -actinin is the AZ191 major component of a nemaline rod13,14. Other Z-disc proteins including myotilin and nebulin are also expressed in rods15,16. Type I fiber predominance and atrophy or hypertrophy are other common features of nemaline myopathy5,12,17. Mutations that cause nemaline myopathy have been identified in-actin (ACTA1),-and-tropomyosin (TPM3andTPM2), troponin T (TNNT1), nebulin (NEB), and cofilin2 (CFL2)18. Mutations in the ryanodine receptor (RYR1)19,20and nebulin (NEB)21have in some cases been found to be <a href=\"https:\/\/www.adooq.com\/az191.html\">AZ191<\/a> responsible for congenital core-rod myopathy. Recently, autosomal dominant nemaline myopathy in two families, Dutch22and Australian-Dutch23, was mapped to a locus on chromosome 15q21-q23 with a combined LOD score of AZ191 10.65 at marker D15S99323and named NEM6 (MIM 609273). Affected individuals in both reported families developed the disease in their infancy or AZ191 childhood and experienced very slowly progressing weakness in the neck and proximal limb muscles with no facial, AZ191 respiratory or distal muscle involvement. Each patient of the Dutch family manifested slowness of movements. Analysis of muscle biopsies in both families showed numerous rods and core-like structures22,23. We describe clinical, myopathological and genetic findings in a Spanish family with four cases fully consistent with published diagnostic criteria for nemaline myopathy5,7. The disease in this family maps to the 15q22. 31 chromosomal locus fully congruent with the region previously identified in the larger Dutch and Australian-Dutch families. == MATERIALS AND METHODS == == The Spanish family == Four patients with myopathy have been identified in a family that originated from Andalucia in Southern Spain (Physique 1). All affected and 7 unaffected family members underwent a clinical study that included general neurologic exam, muscle strength assessment according to the Medical Research Council (MRC) <a href=\"http:\/\/www.edgate.com\/wintergames\/design\/spotlight_sport\/skijump.htm\">Rabbit Polyclonal to MMP-9<\/a> grading scale, serum CK test, and, if affected, nerve conduction testing and concentric needle EMG. Respiratory function assessments and cardiological examination including ECG, 24-hour-Holter monitoring, and echocardiography were performed in individuals II:3 and II:5. == Physique 1. == Pedigree of the Spanish nemaline myopathy family. Filled symbols indicate individuals affected with myopathy; empty symbols represent unaffected family members. Bracketed bars indicate disease-associated haplotype constructed with the use of chromosome 15q marker alleles. The marker order, from centromere to telomere, is usually D15S1033, D15S1036, D15S1507, D15S153. D15S131. == Muscle imaging == Muscle imaging studies were carried out in individuals II:3, II:5 and III:5 at the age of 60, 55 and 34 years, respectively. In patient II:3, transverse and coronal T1-weighted spin-echo (SE) and short-time inversion recovery (STIR) magnetic resonance imaging were.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\ufeffOn ATPase staining, there was type I fiber predominance and hypertrophy; most type II fibers were atrophic. rod-shaped inclusions or nemaline bodies in muscle fibers14. 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