Library

feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Fukuyama type congenital muscular dystrophy (FCMD) is an autosomal recessive severe muscular dystrophy associated with an anomaly of the brain. Twenty–one FCMD families, 13 of them with consanguineous marriages, were analysed by genetic linkage analyses with polymorphic microsatellite markers ...
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 484-491 
    ISSN: 1432-0533
    Keywords: Nemaline myopathy ; Lysosomal enzymes ; Acid phosphatase ; Cathepsins ; Myofibrillar degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four of seven patients with nemaline myopathy had severe, rapidly progressing symptoms. These four showed an increase in acid phosphatase activity in muscle fibers demonstrated by histochemistry and cathepsin B&L activity by biochemical measurement. On electron microscopy, nemaline bodies, occasionally disorganized myofibrils and autophagic vacuoles containing sarcoplasmic debris and glycogen particles were seen. Focal myofibrillar degeneration, through an unknown pathogenetic mechanism, induces an increase in lysosomal enzymes in the skeletal muscles which may be closely correlated with a rapid aggravation of muscle weakness in nemaline myopathy.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 252-257 
    ISSN: 1432-0533
    Keywords: Satellite cell ; Satellite cell replication ; Regeneration ; Bupivacaine ; Bromodeoxyuridine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine how and when the satellite cells are stimulated to replicate in muscle regeneration, the rat soleus muscle was examined chronologically after bupivacaine-induced myonecrosis. Bromodeoxyuridine and desmin-positive mononuclear cells, indicating the start of satellite cell replication, were seen 25 h after bupivacaine treatment when macrophages had already invaded the sarcoplasm of necrotic fiber. These findings suggest that muscle regeneration starts as early as the time at which macrophages begin to scavenge necrotic material. Proliferating myoblasts increased in number, reaching a maximum at 49 h after myonecrosis, and decreased in number 3 days after the myoblasts fused with each other to form myotubes. The satellite cell proliferation after bupivacaine-induced myonecrosis began at almost the same time as in crush injury, and earlier than after muscle transplantation using whole intact or minced muscle fragments. The earlier begining and more rapid regenerating process probably resulted from the preservation of intact satellite cells, blood vessels and peripheral nerves in the bupivacaine-induced myonecrosis.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 252-257 
    ISSN: 1432-0533
    Keywords: Key words Satellite cell ; Satellite cell replication ; Regeneration ; Bupivacaine ; Bromodeoxyuridine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine how and when the satellite cells are stimulated to replicate in muscle regeneration, the rat soleus muscle was examined chronologically after bupivacaine-induced myonecrosis. Bromodeoxyuridine and desmin-positive mononuclear cells, indicating the start of satellite cell replication, were seen 25 h after bupivacaine treatment when macrophages had already invaded the sarcoplasm of necrotic fiber. These findings suggest that muscle regeneration starts as early as the time at which macrophages begin to scavenge necrotic material. Proliferating myoblasts increased in number, reaching a maximum at 49 h after myonecrosis, and decreased in number 3 days after the myoblasts fused with each other to form myotubes. The satellite cell proliferation after bupivacaine-induced myonecrosis began at almost the same time as in crush injury, and earlier than after muscle transplantation using whole intact or minced muscle fragments. The earlier begining and more rapid regenerating process probably resulted from the preservation of intact satellite cells, blood vessels and peripheral nerves in the bupivacaine-induced myonecrosis.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 5
    ISSN: 1432-0533
    Keywords: Muscle necrosis ; Regeneration ; Fibrosis ; Bupivacaine ; Acid anhydride
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We compared the morphologic characteristics of muscle fiber necrosis and subsequent regeneration after injury induced by intramuscular injections of bupivacaine hydrochloride (BPVC) and a variety of solutions at acid and alkaline pH (acetic anhydride, citric acid buffer, and sodium carbonate buffer). After BPVC injection the necrotic muscle fibers were rapidly invaded by phagocytic cells, followed by active regeneration and very little fibrous scar formation. The regenerating muscle fibers increased rapidly in size and attained complete fiber type differentiation and regained their initial fiber diameter within 1 month. Both alkaline and acid solutions induced muscle fiber necrosis followed by regeneration. Fiber necrosis induced by alkaline buffers and acetic anhydride solutions above pH 5.0 produced changes quite similar to that induced by BPVC. However, injection with 0.1 M acetic anhydride at pH below 4.0 resulted in coagulative necrosis of the injured muscle with very little phagocytic infiltration with poor regenerative activity and dense fibrous tissue scarring. Thus, pH 4.0 appears to be the critical pH determining the type of muscle injury and subsequent poor phagocytic and regenerative activities. This model of acidic acetic anhydride injury may lead to the identification of factors which interfere with regeneration and cause fibrous tissue scarring in human muscular dystrophy.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 60 (1983), S. 167-174 
    ISSN: 1432-0533
    Keywords: Muscle necrosis ; Regeneration ; Local anesthetic ; Bupivacaine ; Leupeptin ; Protease inhibitor ; Skinned fibers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A single direct injection of a local anesthetic, 0.5% bupivacaine hydrochloride (BPVC) (Marcaine), into rat soleus and extensor digitorum longus (EDL) muscles produced massive fiber necrosis with extensive phagocytosis followed by rapid regeneration, predominantly in the soleus. Since the sarcoplasmic reticulum (SR) was functionally disturbed by BPVC administration as confirmed by an in vitro study, the sarcolemmal lysis seen in the early phase of degeneration was not assumed to simply results from direct damage to the plasma membrane caused by BPVC. The extracellular fluid containing a high concentration of calcium (Ca) ions then permeated into the sarcoplasm through the defective membrane resulting in hypercontracted myofibrils. Selective damage to the Z-line, an early sign of muscle degeneration, was shown by electron microscopy and SDS gel electrophoresis (preferential loss of α-actinin). Administration of leupeptin, a thiol protease inhibitor, proved to be ineffective in inhibiting the necrotic process, because the BPVC induced muscle fiber breakdown was probably too acute and fulminant to demonstrate the inhibitory effect upon the degenerative process. Well preserved satellite cells, peripheral nerves, and acetylcholinesterase activity, and the absence of fibrous tissue proliferation in this system may be responsible for the extremely rapid regeneration with complete muscle fiber type differentiation. Since the sequence of fiber breakdown induced by BPVC administration was similar to that of progressive muscular dystrophy, this chemical will be one of the most useful tools for studying the pathophysiology of fiber necrosis and regeneration in diseased muscle.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 7
    ISSN: 1432-0533
    Keywords: Acid maltase deficiency ; Japanese quail ; Early morphological change ; Membrane-bound glycogen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The skeletal muscle of Japanese quails with acid maltase deficiency (AMD) was studied morphologically at various developmental stages, from the 16th embryonal day up to 3 months after hatching. Membrane-bound glycogen particles began to appear in the affected skeletal muscle at the 16th embryonal day. In normal embryonic muscles, a certain amount of free glycogen particles was observed but they were not membrane-bound. Therefore, this is the earliest morphological event in the muscle of Japanese quails with AMD. In muscle at 3 weeks after hatching, the initial focal degeneration of myofibrils was recognizable but it was not associated with autophagic vacuoles. Quails with AMD developed muscle weakness and difficulty in lifting their wings at about 3 months after hatching: then numerous autophagic vacuoles were present. The formation of large autophagic vacuoles followed by fiber loss and fatty replacement seemed ot contribute to the progressive muscle weakness. The study of Japanese quail with AMD will greatly facilitate the elucidation of the pathogenetic mechanism and is also a useful model for therapeutic trials in human AMD.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 8
    ISSN: 1432-0533
    Keywords: Dystrophic chicken ; αW fibers ; Intracytoplasmic vacuoles ; Membrane defect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An electron-microscopic study on dystrophic chicken white muscle, posterior latissimus dorsi (PLD), was performed with histochemical identification of three fiber types of βR (red), αR and αW (white) fibers to evaluate the pathophysiology in fiber necrosis. As seen in histochemically stained sections, vacuolar formation in the cytoplasm, an outstanding pathologic feature in chicken dystrophy, was recognized in the αW fibers by electron microscopy. The vacuole was membrane-bound and thought to originate from coalescence or dilatation of extensively proliferated sarcotubular system. There was evidence of a delay in fiber type transformation from αR to αW in dystrophic white muscle, while the initial pathologic event of sarcotubular system proliferation might be expressed only after muscle fibers had attained histochemical characteristics of αW fibers. Localized myofibrillar degeneration was encountered in the vicinity of the vacuole with focal membrane defect. An influx of extracellular fluid through the vacuolated sarcotubular system into the sarcoplasm may activate certain proteases, such as calcium-dependent protease because the extracellular fluid contains high concentration of calcium ion. The activated protease then degrades structural protein, especially Z-line protein, followed by fiber necrosis with phagocytosis.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 58 (1982), S. 279-285 
    ISSN: 1432-0533
    Keywords: Protease inhibitor ; Leupeptin ; Muscular dystrophy ; Dystrophic chicken
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary For the purpose of observing the therapeutic benefit of protease inhibitors for progressive muscular dystrophy, a large quantity of doses of leupeptin of 10 mg/kg/day and 50 mg/kg/day were administered i.p. to male chickens afflicted with hereditary muscular dystrophy (line 413) for 4 months starting on the 7th day ex ovo. No clinical improvement was identified in physical ability as a result of the examination by flip test, and creatine kinase (CK) values. The number of necrotic fibers in the pectoralis superficialis (PS) muscle which is known to be preferentially damaged in dystrophic chicken, did not decrease significantly in the birds treated with 10 mg leupeptin/kg/day (number of necrotic fibers; 47.7/mm2) and 50 mg/kg/day (46.4/mm2) as compared to that of the untreated ones (43.2/mm2). A morphometric analysis of fiber diameter distribution also showed no statistical difference between the treated and untreated birds. In the second group, 10 mg leupeptin/kg and a combination of leupeptin and bestatin of 10 mg/kg each were injected directly into the left lower half of the PS muscle three times a week for 4 months. Necrotic fibers were still present in the injected site, remote area of the left upper PS muscle treated with leupeptin (52.7/mm2), leupeptin and bestatin (52.2/mm2), and contralateral right upper PS muscle (41.6 and 53.5/mm2, respectively). The number of necrotic fibers in treated muscles was again not significantly different from that in untreated dystrophic ones (39.6/mm2). In fiber diameter analysis, no statistical difference was recognized between the treated and untreated dystrophic muscles.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 366-372 
    ISSN: 1432-0533
    Keywords: Cortical dysplasia ; Neuronal migration disorder ; Neurological mutant ; Shaking rat ; Kawasaki (SRK) ; Wistar rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Shaking rat Kawasaki (SRK), a newly discovered neurological mutant rat in the Wistar strain, is described. The abnormalities of SRK rats are transmitted as an autosomal recessive trait. The neurological signs are shaking of the body and an ataxic-paretic gait from day 10 postnatal. The affected rats survive for about 1 month. Macroscopically, the cerebellum is small and frequently the vermis and paraflocculus lacking. The most conspicuous histological finding in the central nervous system is malposition of the neurons in the cerebral cortex, hippocampus and cerebellum. Myelination and synapse formation are intact. Abnormal myelinated fibers are present in the molecular layer of the cerebral cortex and in the central gray matter of the spinal cord. These morphological abnormalities resemble those reported in the reeler mutant mouse. SRK rats are another good animal model of human congenital malformations with neuronal migration disorders.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...