Michael J. Dixon

Missense mutations that cause Van der Woude syndrome and popliteal pterygium syndrome affect the DNA-binding and transcriptional activation functions of IRF6 (2009)

Little, Hayley J., Rorick, Nicholas K., Su, Ling-I, Baldock, Clair, Malhotra, Saimon, Jowitt, Tom, ...

Cleft lip and cleft palate (CLP) are common disorders that occur either as part of a syndrome, where structures other than the lip and palate are affected, or in the absence of other anomalies. Van...

Integration of IRF6 and Jagged2 signalling is essential for controlling palatal adhesion and fusion competence (2009)

Richardson, Rebecca J., Dixon, Jill, Jiang, Rulang, Dixon, Michael J.

In mammals, adhesion and fusion of the palatal shelves are essential mechanisms during the development of the secondary palate; failure of these processes leads to the congenital anomaly, cleft...

Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia (2008)

Barron, Martin J, McDonnell, Sinead T, MacKie, Iain, Dixon, Michael J

Abstract The hereditary dentine disorders, dentinogenesis imperfecta (DGI) and dentine dysplasia (DD), comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine...

The cell adhesion molecule nectin-1 is critical for normal enamel formation in mice (2008)

Barron, Martin J., Brookes, Steven J., Draper, Clare E., Garrod, David, Kirkham, Jennifer, Shore, Roger C., ...

Nectin-1 is a member of a sub-family of immunoglobulin-like adhesion molecules and a component of adherens junctions. In the current study, we have shown that mice lacking nectin-1 exhibit defective...

The Treacher Collins syndrome (TCOF1) gene product is involved in pre-rRNA methylation (2005)

Gonzales, Bianca, Henning, Dale, So, Rolando B., Dixon, Jill, Dixon, Michael J., Valdez, Benigno C.

Treacher Collins syndrome (TCS) is characterized by defects in craniofacial development, which results from mutations in the TCOF1 gene. TCOF1 encodes the nucleolar phosphoprotein treacle, which...

The Treacher Collins syndrome (TCOF1) gene product is involved in pre-rRNA methylation (2005)

Gonzales, Bianca, Henning, Dale, So, Rolando B., Dixon, Jill, Dixon, Michael J., Valdez, Benigno C.

Treacher Collins syndrome (TCS) is characterized by defects in craniofacial development that result from mutations in the TCOF1 gene. TCOF1 encodes the nucleolar phosphoprotein treacle which...

DNA elements important for CAG{middle dot}CTG repeat thresholds in Saccharomyces cerevisiae (2004)

Dixon, Michael J., Lahue, Robert S.

Trinucleotide repeat (TNR) instability is of interest because of its central role in human diseases such as Huntington’s and its unique genetic features. One distinctive characteristic of TNR...

Mutation of the signal peptide region of the bicistronic gene DSPP affects translocation to the endoplasmic reticulum and results in defective dentine biomineralization (2002)

Rajpar, M. Helen, Koch, Martin J., Davies, Robin M., Mellody, Kieran T., Kielty, Cay M., Dixon, Michael J.

Dentine dysplasia type II is an autosomal dominant disorder in which mineralization of the dentine of the primary teeth is abnormal. On the basis of the phenotypic overlap between, and shared...

Spectrum of FOXL2 gene mutations in blepharophimosis-ptosis-epicanthus inversus (BPES) families demonstrates a genotype-phenotype correlation (2001)

De Baere, Elfride, Dixon, Michael J., Small, Kent W., Jabs, Ethylin W., Leroy, Bart P., Devriendt, Koenraad, ...

Mutations in FOXL2, a forkhead transcription factor gene, have recently been shown to cause blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) types I and II, a rare genetic disorder. In...

Mutation of the gene encoding the enamel-specific protein, enamelin, causes autosomal-dominant amelogenesis imperfecta (2001)

Rajpar, M. Helen, Harley, Kathryn, Laing, Chris, Davies, Robin M., Dixon, Michael J.

Amelogenesis imperfecta (AI) is a group of inherited defects of dental enamel formation that shows both clinical and genetic heterogeneity. To date, mutations in the gene encoding amelogenin have...

Mutation of the gene encoding fibrillin-2 results in syndactyly in mice (2001)

Chaudhry, Shazia S., Gazzard, James, Baldock, Clair, Dixon, Jill, Rock, Matthew J., Skinner, Gail C., ...

Fibrillins are large, cysteine-rich glycoproteins that form microfibrils and play a central role in elastic fibrillogenesis. Fibrillin-1 and fibrillin-2, encoded by FBN1 on chromosome 15q21.1 and...

Increased levels of apoptosis in the prefusion neural folds underlie the craniofacial disorder, Treacher Collins syndrome (2000)

Dixon, Jill, Brakebusch, Cord, Fässler, Reinhard, Dixon, Michael J.

Treacher Collins syndrome (TCS) is an autosomal dominant disorder of human craniofacial development that results from loss-of-function mutations in the gene TCOF1. Although this gene has been...

Mapping of dentin-specific acidic phosphoprotein and integrin-binding sialoprotein in sheep defines an inversion breakpoint with respect to human chromosome 4Q (1999)

Lumsden,Joanne M., Lord,Eric A., Hirst,Karen L., Dixon,Michael J., Montgomery,Grant W.

Genes from sheep chromosome 6 map to human chromosome 4 in the region extending from 4p16 to 4q26. However, there is an inversion of gene order in the central portion of the chromosome with one...

An Overdue Post-Cold War Army Structure (1998)

Dixon, Michael J.

Over the past five years, the United States Army (USA) has taken significant actions to increase its readiness and relevance to Joint Force Commander (JFC). The Army has rapidly aligned itself with...

Definition of the blepharophimosis, ptosis, epicanthus inversus syndrome critical region at chromosome 3q23 based on the analysis of chromosomal anomalies (1995)

Lawson, Claire T., Toomes, Carmel, Fryer, Alan, Carette, Martin J.M., Taylor, G.Malcolm, Fukushima, Yoshimitsu, ...

Blepharophimosis syndrome (BPES) is an autosomal dominant disorder of craniofacial development, the features of which are small palpebral fissures (blepharophimosis), drooping eyelids (ptosis) and a...

A combined genetic and radiation hybrid map surrounding the Treacher Collins syndrome locus on chromosome 5q (1993)

Loftus, Stacie K., Edwards, Sara J., Scherpbier-Heddema, Titia, Buetow, Kenneth H., Wasmuth, John J., Dixon, Michael J.

The distal region of chromosome 5q contains a large number of genes, including those implicated in a variety of Mendelian disorders. One of these, Treacher Collins syndrome (TCOF1), is an autosomal...

Genetic and physical mapping of the Treacher Collins syndrome locus: refinement of the localization to chromosome 5q32-33.2 (1992)

Dixon, Michael J., Dixon, Jill, Raskova, Dagmar, Le Beau, Michelle M., Williamson, Robert, Kinger, Katherine, ...

Treacher Collins syndrome (TCOF1) is an autosomal dominant disorder of craniofacial development, the locus for which has been chromosomally localized to 5q31-34. We have isolated four hypervarlable...

Characterization of the Nucleolar Gene Product, Treacle, in Treacher Collins Syndrome

Isaac, Cynthia, Marsh, Karen L., Paznekas, William A., Dixon, Jill, Dixon, Michael J., Jabs, Ethylin Wang, ...

Treacher Collins syndrome (TCS) is an autosomal dominant disorder of craniofacial development caused by mutations in the gene TCOF1. Its gene product, treacle, consists mainly of a central repeat...

A Preliminary Gene Map for the Van der Woude Syndrome Critical Region Derived from 900 kb of Genomic Sequence at 1q32–q41

Schutte, Brian C., Bjork, Bryan C., Coppage, Kevin B., Malik, Margaret I., Gregory, Simon G., Scott, Deborah J., ...

Van der Woude syndrome (VWS) is a common form of syndromic cleft lip and palate and accounts for ∼2% of all cleft lip and palate cases. Distinguishing characteristics include cleft lip with or...

DNA elements important for CAG·CTG repeat thresholds in Saccharomyces cerevisiae

Dixon, Michael J., Lahue, Robert S.

Trinucleotide repeat (TNR) instability is of interest because of its central role in human diseases such as Huntington’s and its unique genetic features. One distinctive characteristic of TNR...

The Treacher Collins syndrome (TCOF1) gene product is involved in ribosomal DNA gene transcription by interacting with upstream binding factor

Valdez, Benigno C., Henning, Dale, So, Rolando B., Dixon, Jill, Dixon, Michael J.

Treacher Collins syndrome (TCS) is an autosomal dominant disorder characterized by an abnormality of craniofacial development that arises during early embryogenesis. TCS is caused by mutations in the...

Identification of RTG2 as a modifier gene for CTG*CAG repeat instability in Saccharomyces cerevisiae.

Bhattacharyya, Saumitri, Rolfsmeier, Michael L, Dixon, Michael J, Wagoner, Kara, Lahue, Robert S

Trinucleotide repeats (TNRs) undergo frequent mutations in families affected by TNR diseases and in model organisms. Much of the instability is conferred in cis by the sequence and length of the...

Characterization of the Nucleolar Gene Product, Treacle, in Treacher Collins Syndrome

Isaac, Cynthia, Marsh, Karen L., Paznekas, William A., Dixon, Jill, Dixon, Michael J., Jabs, Ethylin Wang, ...

Treacher Collins syndrome (TCS) is an autosomal dominant disorder of craniofacial development caused by mutations in the gene TCOF1. Its gene product, treacle, consists mainly of a central repeat...

A Preliminary Gene Map for the Van der Woude Syndrome Critical Region Derived from 900 kb of Genomic Sequence at 1q32–q41

Schutte, Brian C., Bjork, Bryan C., Coppage, Kevin B., Malik, Margaret I., Gregory, Simon G., Scott, Deborah J., ...

Van der Woude syndrome (VWS) is a common form of syndromic cleft lip and palate and accounts for ∼2% of all cleft lip and palate cases. Distinguishing characteristics include cleft lip with or...

DNA elements important for CAG·CTG repeat thresholds in Saccharomyces cerevisiae

Dixon, Michael J., Lahue, Robert S.

Trinucleotide repeat (TNR) instability is of interest because of its central role in human diseases such as Huntington’s and its unique genetic features. One distinctive characteristic of TNR...

The Treacher Collins syndrome (TCOF1) gene product is involved in ribosomal DNA gene transcription by interacting with upstream binding factor

Valdez, Benigno C., Henning, Dale, So, Rolando B., Dixon, Jill, Dixon, Michael J.

Treacher Collins syndrome (TCS) is an autosomal dominant disorder characterized by an abnormality of craniofacial development that arises during early embryogenesis. TCS is caused by mutations in the...

Identification of RTG2 as a modifier gene for CTG*CAG repeat instability in Saccharomyces cerevisiae.

Bhattacharyya, Saumitri, Rolfsmeier, Michael L, Dixon, Michael J, Wagoner, Kara, Lahue, Robert S

Trinucleotide repeats (TNRs) undergo frequent mutations in families affected by TNR diseases and in model organisms. Much of the instability is conferred in cis by the sequence and length of the...

Tcof1/Treacle is required for neural crest cell formation and proliferation deficiencies that cause craniofacial abnormalities

Dixon, Jill, Jones, Natalie C., Sandell, Lisa L., Jayasinghe, Sachintha M., Crane, Jennifer, Rey, Jean-Philippe, ...

Neural crest cells are a migratory cell population that give rise to the majority of the cartilage, bone, connective tissue, and sensory ganglia in the head. Abnormalities in the formation,...

The cell adhesion molecule nectin-1 is critical for normal enamel formation in mice

Barron, Martin J., Brookes, Steven J., Draper, Clare E., Garrod, David, Kirkham, Jennifer, Shore, Roger C., ...

Nectin-1 is a member of a sub-family of immunoglobulin-like adhesion molecules and a component of adherens junctions. In the current study, we have shown that mice lacking nectin-1 exhibit defective...

Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia

Barron, Martin J, McDonnell, Sinead T, MacKie, Iain, Dixon, Michael J

The hereditary dentine disorders, dentinogenesis imperfecta (DGI) and dentine dysplasia (DD), comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine structure...

Integration of IRF6 and Jagged2 signalling is essential for controlling palatal adhesion and fusion competence

Richardson, Rebecca J., Dixon, Jill, Jiang, Rulang, Dixon, Michael J.

In mammals, adhesion and fusion of the palatal shelves are essential mechanisms during the development of the secondary palate; failure of these processes leads to the congenital anomaly, cleft...

Missense mutations that cause Van der Woude syndrome and popliteal pterygium syndrome affect the DNA-binding and transcriptional activation functions of IRF6

Little, Hayley J., Rorick, Nicholas K., Su, Ling-I, Baldock, Clair, Malhotra, Saimon, Jowitt, Tom, ...

Cleft lip and cleft palate (CLP) are common disorders that occur either as part of a syndrome, where structures other than the lip and palate are affected, or in the absence of other anomalies. Van...