W. J. Kimberling

Publication List Details

Period

1995 - 1996

Number

31

Co-Authors

Novel mutations in the connexin 26 gene (GJB2) that cause autosomal recessive (DFNB1) hearing loss.

Kelley, P M, Harris, D J, Comer, B C, Askew, J W, Fowler, T, Smith, S D, ...

Mutations in the connexin 26 (Cx26) gene (GJB2) are associated with the type of autosomal recessive nonsyndromic neurosensory deafness known as "DFNB1." Studies indicate that DFNB1 (13q11-12) causes...

Genomewide search and genetic localization of a second gene associated with autosomal dominant branchio-oto-renal syndrome: clinical and genetic implications.

Kumar, S, Deffenbacher, K, Marres, H A, Cremers, C W, Kimberling, W J

Branchio-oto-renal (BOR) syndrome is characterized by ear malformations, cervical fistulas, hearing loss, and renal anomalies. It is an autosomal dominant disorder with variable clinical...

CDH23 Mutation and Phenotype Heterogeneity: A Profile of 107 Diverse Families with Usher Syndrome and Nonsyndromic Deafness

Astuto, L. M., Bork, J. M., Weston, M. D., Askew, J. W., Fields, R. R., Orten, D. J., ...

Usher syndrome type I is characterized by congenital hearing loss, retinitis pigmentosa (RP), and variable vestibular areflexia. Usher syndrome type ID, one of seven Usher syndrome type I genetic...

Novel mutations in the connexin 26 gene (GJB2) that cause autosomal recessive (DFNB1) hearing loss.

Kelley, P M, Harris, D J, Comer, B C, Askew, J W, Fowler, T, Smith, S D, ...

Mutations in the connexin 26 (Cx26) gene (GJB2) are associated with the type of autosomal recessive nonsyndromic neurosensory deafness known as "DFNB1." Studies indicate that DFNB1 (13q11-12) causes...

Genomewide search and genetic localization of a second gene associated with autosomal dominant branchio-oto-renal syndrome: clinical and genetic implications.

Kumar, S, Deffenbacher, K, Marres, H A, Cremers, C W, Kimberling, W J

Branchio-oto-renal (BOR) syndrome is characterized by ear malformations, cervical fistulas, hearing loss, and renal anomalies. It is an autosomal dominant disorder with variable clinical...

Localization of spherocytosis to chromosome 8 or 12 and report of a family with spherocytosis and a reciprocal translocation.

Kimberling, W J, Fulbeck, T, Dixon, L, Lubs, H A

Significant linkage was found between spherocytosis and a translocation involving the short arms of chromosomes 8 and 12. The gene for spherocytosis, therefore, is likely either very close to the...

Myosin VIIA mutation screening in 189 Usher syndrome type 1 patients.

Weston, M. D., Kelley, P. M., Overbeck, L. D., Wagenaar, M., Orten, D. J., Hasson, T., ...

Usher syndrome type 1b (USH1B) is an autosomal recessive disorder characterized by congenital profound hearing loss, vestibular abnormalities, and retinitis pigmentosa. The disorder has recently been...

Refining the region of branchio-oto-renal syndrome and defining the flanking markers on chromosome 8q by genetic mapping.

Kumar, S., Kimberling, W. J., Connolly, C. J., Tinley, S., Marres, H. A., Cremers, C. W.

Branchio-oto-renal syndrome (BOR) is an autosomal dominant disorder associated with external-, middle-, and inner-ear malformations, branchial cleft sinuses, cervical fistulas, mixed hearing loss,...

Genetic heterogeneity of Usher syndrome type II.

Pieke Dahl, S, Kimberling, W J, Gorin, M B, Weston, M D, Furman, J M, Pikus, A, ...

Usher syndrome is an autosomal recessive disorder characterised by retinitis pigmentosa and congenital sensorineural hearing loss. A gene for Usher syndrome type II (USH2) has been localised to...

Exclusion of autosomal dominant polycystic kidney disease type II (ADPKD2) from 160 cM of chromosome 1.

Kumar, S, Kimberling, W J, Gabow, P A, Shugart, Y Y, Pieke-Dahl, S

Autosomal dominant polycystic kidney disease is a heritable disorder and recent studies have shown genetic heterogeneity, with some, but not all, families showing linkage with markers on chromosome...

Phenotypic variation in the familial atypical multiple mole-melanoma syndrome (FAMMM).

Lynch, H T, Fusaro, R M, Albano, W A, Pester, J, Kimberling, W J, Lynch, J F

The familial atypical multiple mole-melanoma syndrome (FAMMM) is characterised by an autosomal dominantly inherited susceptibility to multiple atypical moles which show variable colouration ranging...

Familial atypical multiple mole-melanoma (FAMMM) syndrome: segregation analysis.

Lynch, H T, Fusaro, R M, Kimberling, W J, Lynch, J F, Danes, B S

Genetic analysis was performed on four kindreds with clinical and pathological verification of the FAMMM syndrome. There were 80 affected or at risk members in these families. A segregation ratio of...

Breast cancer, genetics, and age at first pregnancy.

Lynch, H T, Albano, W A, Layton, M A, Kimberling, W J, Lynch, J F

Hereditary breast cancer shows a distinctive natural history characterised by an earlier age of onset, excess bilaterality, vertical transmission, heterogeneous tumour associations, and improved...

Genetic heterogeneity of Usher syndrome type II in a Dutch population.

Pieke-Dahl, S, Van Aarem, A, Dobin, A, Cremers, C W, Kimberling, W J

The Usher syndromes are a group of autosomal recessive disorders characterised by retinitis pigmentosa (RP) with congenital, stable (non-progressive) sensorineural hearing loss. Profound deafness,...