The Muscular Dystrophies and Related Disorders

Furukawa, Tatsuo

doi:10.1001/jama.1978.03280430070022

Cited by 8 publications

(1 citation statement)

References 73 publications

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“…The product of the gene -protein-dystrophin (D) was described in 1987 y. E. Hoffman [4]. Now there is much information concerning of D, which don't exist isolated, it forms tightly associated complexes [1][2][3][4][5][6][7][8][9][10]. The dystroglucoprotein complex -DGC-the most studying, plays a mechanical function in stabilizing the sarcolemma against stresses during muscles contraction; role scaffold in neuromuscular junctions; the general function is the connection the cytoskeleton to the extracellular matrix.…”

Section: Introductionmentioning

confidence: 99%

A New Hypothesis of Duchenne Muscular Dystrophy

Grinio¹

2021

AJPN

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Duchenne Muscular Dystrophy is the product the mutation the gene-dystrophine and appearing defective protein dystropnin. Its function is unclear, suppose protection membranes during the contraction-relax skeletal muscles. The cytoplasmic dystrophin acts as the complexes with different proteins inside and around membranes, which are placing in skeletal muscls, heart, different regions brain, internal organs, their function unclear. The high activity enzyme creatin-kinasa in the patients blood is the known fact which is usually explain damage of the process contraction-relax skeletal muscles. The surprising activity enzyme -23 000 M E was found by author at large families with typical pedigree where have some patients and some boys without signs muscle weakness. There were 4 boys 12-22 months life during forming walk, later these boys were diagnosed as Duchenne Muscular Dystrophy patients. Such activity I did not find in literature. Usually the highest activity enzyme 10 000-12 000 ME. This fact testify the simultaneously damage many membranes on large territory, it permits suppose organized damage membranes. Author believe all complexes normal dystrophines may work as one System, beginning learn walk and finishing as age myopathy after 60 years. Suppose the System was ancient and appeared when movements become intensive and one gene utrophine/dystrophine turned out in two genes: utrophin and dystrophin. Dystrophin signal communication is known, but its investigation has begun and showed complex signaling pathways. It is possible to suppose that the first stage of the disease is damage signaling ways. Damage homeostasis and membranes is the second stage. There are deep changes of metabolism: decreasing true muscle proteins, phospholipids, increasing hormones, appearing hyper aminoaciduria. Apoptosis -the three stage of the disease and general destructive factor which turn out pathologic process to the fatal end. Apoptosis hinder all tryings organism to interrupt pathological process and therapeutic trying. Apoptosis can not stop once it began. All three stages have place in preclinical time. The clinic symptoms express destruction more the half skeletal muscles, severe damage metabolism, damage system protection membranes and can not be onset of the disease. The important problem of the disease -studying interaction dystrophin-complexes with membranes during physical stress, signaling ways. Two factors determinate rapid course: damage D-System and apoptosis.

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Section: Introductionmentioning

confidence: 99%

A New Hypothesis of Duchenne Muscular Dystrophy

Grinio¹

2021

AJPN

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Screening of Antiserotoninergic Drugs With the Genetically Dystrophic Chicken

Hudecki¹,

Pollina²,

Bhargava³

et al. 1980

Archives of Neurology

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Delayed functional disability in dystrophic chickens receiving chemotherapy

Hudecki¹,

Pollina²,

Bhargava³

et al. 1979

Muscle and Nerve

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Line 413 early-onset, genetically homozygous dystrophic chickens were given twice-daily intraperitoneal injections of the antiserotoninergic drug cinanserin, alone or in combination with methysergide. Other trials consisted of penicillamine treatment in combination with either methysergide or cyproheptadine. Chemotherapy significantly prolonged the righting ability of treated dystrophic chickens, as measured by a periodic standardized flip-test procedure. Plasma creatine kinase activities were not affected by any of the various drug treatments. However, the blood serotonin levels of the dystrophic chickens (grand mean 1.47 microgram serotonin/ml blood) were found to be significantly higher (p less than 0.001) than those in the corresponding normal chickens (0.99 microgram/ml). This finding may partially account for the antiserotoninergic drug enhancement in righting ability that was demonstrated in the drug-treated dystrophic chickens.

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The Muscular Dystrophies and Related Disorders

Cited by 8 publications

References 73 publications

A New Hypothesis of Duchenne Muscular Dystrophy

A New Hypothesis of Duchenne Muscular Dystrophy

Screening of Antiserotoninergic Drugs With the Genetically Dystrophic Chicken

Delayed functional disability in dystrophic chickens receiving chemotherapy

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