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Reversal of brain atrophy with biotin treatment in biotinidase deficiency.
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Two children with biotinidase deficiency presented with seizures at 2 months of age. The first child had a fluctuating course with continual developmental progress and cessation of seizures despite symptoms of chronic neurologic dysfunction until he was diagnosed at 17 months. The second child had a
Profligate biotin synthesis in α-proteobacteria - a developing or degenerating regulatory system?
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Biotin (vitamin H) is a key enzyme cofactor required in all three domains of life. Although this cofactor was discovered over 70 years ago and has long been recognized as an essential nutrient for animals, our knowledge of the strategies bacteria use to sense biotin demand is very limited. The
Biotin levels in plasma of patients with Friedreich ataxia and other spinocerebellar degenerations.
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Attenuation of hepatotoxicity and oxidative stress in diabetes STZ-induced type 1 by biotin in Swiss albino mice.
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Diabetes mellitus is one of the major health problems. This study was designed to investigate the effect of biotin to regulate blood glucose level, reduced toxicity and oxidative stress in liver of diabetic mice STZ-induced type 1. Male mice were divided into three groups, the first one served as
Contribution of Borneolum syntheticum to the intervention effect of Liuwei Dihuang Pill () on experimental retinal degeneration.
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OBJECTIVE
To observe the contribution of Borneolum syntheticum to the intervention effect of Liuwei Dihuang Pill (, LDP) on experimental retinal degeneration, and initially investigate the mechanism of Borneolum syntheticum as meridian-lead-in drug.
METHODS
A total of 180 sodium iodateinduced
Neurological deficits in mice with profound biotinidase deficiency are associated with demylination and axonal degeneration.
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Biotinidase deficiency is an autosomal recessively inherited disorder characterized by neurological and cutaneous abnormalities. We have developed a transgenic knock-out mouse with biotinidase deficiency to better understand aspects of pathophysiology and natural history of the disorder in humans.
Astrocytes and microglial cells incorporate degenerating fibers following entorhinal lesion: a light, confocal, and electron microscopical study using a phagocytosis-dependent labeling technique.
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Entorhinal lesion leads to anterograde degeneration of perforant path fibers in their main termination zone in the outer molecular layers of the dentate gyrus. Concomitantly, astrocytes become hypertrophic, and microglial cells alter their phenotype, suggesting participation in anterograde
Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity.
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Three siblings presented in early childhood with central-nervous-system (CNS) dysfunction, candida dermatitis, keratoconjunctivitis, and alopecia. Two were studied immunologically and had absent delayed-hypersensitivity skin-test responses and absent in-vitro lymphocyte responses to candida antigen.
A new model of retinal photoreceptor cell degeneration induced by a chemical hypoxia-mimicking agent, cobalt chloride.
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Retinal photoreceptor cell degeneration was induced by cobalt chloride, a chemical hypoxia-mimicking agent in rodents. Time course and dose-response of photoreceptor cell degeneration in mouse retina after intravitreal injection of cobalt chloride were examined by conventional histological analysis
Cystic medial degeneration of the aorta is associated with p53 accumulation, Bax upregulation, apoptotic cell death, and cell proliferation.
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OBJECTIVE
To address a potential role for p53, Bcl2 associated protein X (Bax), and apoptosis in the processes associated with cell turnover during cystic medial degeneration (CMD) of the aorta.
METHODS
Histochemical, immunohistochemical, biochemical, and morphometric methods were used to assess the
Histology and cellular kinetics of prostatic atrophy.
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This study addresses two issues regarding prostatic atrophy: (1) the histologic features of atrophy as seen on needle biopsy results and how they affect the diagnosis of atrophy and (2) the cellular kinetics of atrophy and what it suggests about the mechanism of atrophy. We reviewed hematoxylin and
Anabolic effects of Peniel 2000, a peptide that regulates TGF-β1 signaling on intervertebral disc degeneration.
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METHODS
An in vitro study with bovine intervertebral disc (IVD) cells and an in vivo study with a rabbit disc degeneration model on the extracellular matrix metabolism by a biglycan-derived peptide (Peniel 2000; P2K).
OBJECTIVE
To investigate the mechanism for P2K-induced increases in extracellular
Inherited retinal degeneration and apoptosis in mutant zebrafish.
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The mechanism of retinal cell death was studied in mutant zebrafish (Danio rerio) which undergo inherited degeneration of the retina and the brain. The shrunken head (shr(m33)) mutation was isolated as part of a large scale mutagenesis screen. The yellow head (yhd) mutation arose spontaneously among
A clinicopathological observation of Nyssen-van Bogaert syndrome with second motor neuron degeneration: two distinct clinical entities.
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OBJECTIVE
To report a novel clinicopathological observation of Nyssen-van Bogaert syndrome. To compare this observation with those previously reported. To discuss the nosological entity of this syndrome. To define the exact etiopathogenic mechanism of the neurogenic amyotrophy occurring at the late
Immunohistochemical analysis of the distribution of MyoD1 in muscle biopsies of primary myopathies and neurogenic atrophy.
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The expression of the myogenic determination gene MyoD1 plays a primary role in the commitment of primitive mesenchymal cells to a striated muscle lineage and is down-regulated during later stages of differentiation. To determine the potential role of this gene in myopathic conditions, we examined
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