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Mostrando 81 - 100 Resultados de 1,763 Para Buscar '"Usher"', tiempo de consulta: 0.22s Limitar resultados
  1. 81
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    Retinal Gene Therapy for Usher Syndrome: Current Developments, Challenges, and Perspectives
  2. 82
    “…Adhesive pili assembled through the chaperone–usher pathway are hair-like appendages that mediate host tissue colonization and biofilm formation of Gram-negative bacteria(1–3). …”
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    Archaic chaperone–usher pili self-secrete into superelastic zigzag springs
  3. 83
    “…After negative results in an Usher syndrome panel, targeted genetic testing revealed compound heterozygous mutations in PEX6. …”
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    PEX6 Mutations in Peroxisomal Biogenesis Disorders: An Usher Syndrome Mimic
  4. 84
    “…Fimbriae assembled by the Chaperone Usher pathway (CUP) are widespread within the Enterobacteriaceae, and their expression is tightly regulated by specific environmental stimuli. …”
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    Diversity in Genetic Regulation of Bacterial Fimbriae Assembled by the Chaperone Usher Pathway
  5. 85
    “…PURPOSE: To study the prevalence, level, and nature of sleep problems and fatigue experienced by Usher syndrome type 2a (USH2a) patients. DESIGN: Cross-sectional study. …”
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    Evaluation of Sleep Quality and Fatigue in Patients with Usher Syndrome Type 2a
  6. 86
    “…In more than half of the cases, Usher syndrome (USH) is the origin of these double defects. …”
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    Identification of candidate regions for a novel Usher syndrome type II locus
  7. 87
    “…Here we show developmental expression of these Usher proteins in afferent spiral ganglion neurons and hair cell synapses. …”
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    Role for a Novel Usher Protein Complex in Hair Cell Synaptic Maturation
  8. 88
    “…Usher syndrome (USH), the leading cause of hereditary combined hearing and vision loss, is characterized by sensorineural deafness and progressive retinal degeneration. …”
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    Complexes of Usher proteins preassemble at the endoplasmic reticulum and are required for trafficking and ER homeostasis
  9. 89
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    Pakistan Abolishes Kidney Market and Ushers in a New Era of Ethical Transplantation
  10. 90
    “…The chaperone-usher (CU) pathway assembles organelles termed pili or fimbriae in Gram-negative bacteria. …”
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    The Pilus Usher Controls Protein Interactions via Domain Masking and is Functional as an Oligomer
  11. 91
    “…These pili, assembled by the chaperone-usher pathway, are polymers of pilus subunits assembling into two parts: a thin, short tip fibrillum at the top, mounted on a long pilus rod. …”
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    Structure of a Chaperone-Usher Pilus Reveals the Molecular Basis of Rod Uncoiling
  12. 92
    “…Usher Syndrome (USH) is a rare disease with hearing loss, retinitis pigmentosa and, sometimes, vestibular dysfunction. …”
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    Accelerated age-related olfactory decline among type 1 Usher patients
  13. 93
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    Correction: Allosteric signalling in the outer membrane translocation domain of PapC usher
  14. 94
    “…Usher syndrome type 1 (USH1) causes combined hearing and sight defects, but how mutations in USH1 genes lead to retinal dystrophy in patients remains elusive. …”
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    Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment
  15. 95
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    Indian Society of Anaesthesiologists cardiopulmonary resuscitation guidelines: Ushering in a new initiative
  16. 96
    “…Here, we examine the diversity of usher proteins across a wide range of extraintestinal (ExPEC) and enteropathogenic (EPEC) Escherichia coli, and further focus on a so far undescribed chaperone–usher system, with this usher referred to as UshC. …”
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    Constraints on lateral gene transfer in promoting fimbrial usher protein diversity and function
  17. 97
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    Handover mechanism of the growing pilus by the bacterial outer membrane usher FimD
  18. 98
    “…BACKGROUND: Usher syndrome, the most common form of inherited deaf-blindness, is unlike many other forms of syndromic hereditary hearing loss in that the extra aural clinical manifestations are also detrimental to communication. …”
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    Novel Usher syndrome pathogenic variants identified in cases with hearing and vision loss
  19. 99
    “…Usher syndrome has been historically categorized into one of three classical types based on the patient phenotype. …”
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    Vestibular phenotype‐genotype correlation in a cohort of 90 patients with Usher syndrome
  20. 100
    “…PURPOSE: To describe the molecular epidemiology of nonsyndromic retinitis pigmentosa (RP) and Usher syndrome (US) in Italian patients. METHODS: A total of 591 probands (315 with family history and 276 sporadics) were analyzed. …”
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    Molecular Epidemiology in 591 Italian Probands With Nonsyndromic Retinitis Pigmentosa and Usher Syndrome
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