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1.
Proc Natl Acad Sci U S A ; 97(16): 8815-21, 2000 Aug 01.
Article in English | MEDLINE | ID: mdl-10922040

ABSTRACT

By exploiting the ability of Pseudomonas aeruginosa to infect a variety of vertebrate and nonvertebrate hosts, we have developed model systems that use plants and nematodes as adjuncts to mammalian models to help elucidate the molecular basis of P. aeruginosa pathogenesis. Our studies reveal a remarkable degree of conservation in the virulence mechanisms used by P. aeruginosa to infect hosts of divergent evolutionary origins.


Subject(s)
Arabidopsis/microbiology , Pseudomonas aeruginosa/pathogenicity , Virulence , Animals , Biological Evolution , Burns/microbiology , Mice , Plants
2.
Ann Neurol ; 41(3): 314-25, 1997 Mar.
Article in English | MEDLINE | ID: mdl-9066352

ABSTRACT

Congenital fibrosis of the extraocular muscles is an autosomal dominant congenital disorder characterized by bilateral ptosis, restrictive external ophthalmoplegia with the eyes partially or completely fixed in an infraducted (downward) and strabismic position, and markedly limited and aberrant residual eye movements. It has been generally thought that these clinical abnormalities result from myopathic fibrosis of the extraocular muscles. We describe the intracranial and orbital pathology of 1 and the muscle pathology of 2 other affected members of a family with chromosome 12-linked congenital fibrosis of the extraocular muscles. There is an absence of the superior division of the oculomotor nerve and its corresponding alpha motor neurons, and abnormalities of the levator palpebrae superioris and rectus superior (the muscles innervated by the superior division of the oculomotor nerve). In addition, increased numbers of internal nuclei and central mitochondrial clumping are found in other extraocular muscles, suggesting that the muscle pathology extends beyond the muscles innervated by the superior division of cranial nerve III. This report presents evidence that congenital fibrosis of the extraocular muscles results from an abnormality in the development of the extraocular muscle lower motor neuron system.


Subject(s)
Abnormalities, Multiple/diagnosis , Oculomotor Muscles/abnormalities , Oculomotor Nerve/abnormalities , Ophthalmoplegia/congenital , Abnormalities, Multiple/genetics , Adult , Aged , Amblyopia/rehabilitation , Biopsy , Brain/pathology , Child, Preschool , Chromosome Mapping , Chromosomes, Human, Pair 12 , Female , Fibrosis , Humans , Infant, Newborn , Male , Muscle, Skeletal/pathology , Oculomotor Muscles/pathology , Pedigree , Strabismus/surgery
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