Bernice E. Morrow

Tbx1and Brn4regulate retinoic acid metabolic genes during cochlear morphogenesis (2009)

Braunstein, Evan M, Monks, Dennis C, Aggarwal, Vimla S, Arnold, Jelena S, Morrow, Bernice E

Abstract Background In vertebrates, the inner ear is comprised of the cochlea and vestibular system, which develop from the otic vesicle. This process is regulated via inductive interactions from...

Hominoid lineage specific amplification of low-copy repeats on 22q11.2 (LCR22s) associated with velo-cardio-facial/digeorge syndrome (2007)

Babcock, Melanie, Yatsenko, Svetlana, Hopkins, Janet, Brenton, Matthew, Cao, Qing, De Jong, Pieter, ...

Segmental duplications or low-copy repeats (LCRs) constitute ∼5% of the sequenced portion of the human genome and are associated with many human congenital anomaly disorders. The low-copy repeats...

AT-rich repeats associated with chromosome 22q11.2 rearrangement disorders shape human genome architecture on Yq12 (2007)

Babcock, Melanie, Yatsenko, Svetlana, Stankiewicz, Pawel, Lupski, James R., Morrow, Bernice E.

Low copy repeats (LCRs; segmental duplications) constitute ∼5% of the sequenced human genome. Nonallelic homologous recombination events between LCRs during meiosis can lead to chromosomal...

Dissection of Tbx1 and Fgf interactions in mouse models of 22q11DS suggests functional redundancy (2006)

Aggarwal, Vimla S., Liao, Jun, Bondarev, Alexei, Schimmang, Thomas, Lewandoski, Mark, Locker, Joseph, ...

The 22q11 deletion syndrome (22q11DS) is characterized by abnormal development of the pharyngeal apparatus. Mouse genetic studies have identified Tbx1 as a key gene in the etiology of the syndrome,...

Tissue specific roles of Tbx1 in the development of the outer, middle and inner ear, defective in 22q11DS patients (2006)

Arnold, Jelena S., Braunstein, Evan M., Ohyama, Takahiro, Groves, Andrew K., Adams, Joe C., Brown, M. Christian, ...

Most 22q11.2 deletion syndrome (22q11DS) patients have middle and outer ear anomalies while some have inner ear malformations. Tbx1, a gene hemizygously deleted in 22q11DS patients and required for...

Dissection of Tbx1 and Fgf interactions in mouse models of 22q11DS suggests functional redundancy (2006)

Aggarwal, Vimla S., Liao, Jun, Bondarev, Alexei, Schimmang, Thomas, Lewandoski, Mark, Locker, Joseph, ...

The 22q11 deletion syndrome (22q11DS) is characterized by abnormal development of the pharyngeal apparatus. Mouse genetic studies have identified Tbx1 as a key gene in the etiology of the syndrome,...

Tissue-specific roles of Tbx1 in the development of the outer, middle and inner ear, defective in 22q11DS patients (2006)

Arnold, Jelena S., Braunstein, Evan M., Ohyama, Takahiro, Groves, Andrew K., Adams, Joe C., Brown, M. Christian, ...

Most 22q11.2 deletion syndrome (22q11DS) patients have middle and outer ear anomalies, whereas some have inner ear malformations. Tbx1, a gene hemizygously deleted in 22q11DS patients and required...

Traffic of genetic information between segmental duplications flanking the typical 22q11.2 deletion in velo-cardio-facial syndrome/DiGeorge syndrome (2005)

Pavlicek, Adam, House, Reniqua, Gentles, Andrew J., Jurka, Jerzy, Morrow, Bernice E.

Velo-cardio-facial syndrome/DiGeorge syndrome results from unequal crossing-over events between two 240-kb low-copy repeats termed LCR22 (LCR22-2 and LCR22-4) on Chromosome 22q11.2, comprised of...

Full spectrum of malformations in velo-cardio-facial syndrome/DiGeorge syndrome mouse models by altering Tbx1 dosage (2004)

Liao, Jun, Kochilas, Lazaros, Nowotschin, Sonja, Arnold, Jelena S., Aggarwal, Vimla S., Epstein, Jonathan A., ...

Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS) is associated with de novo hemizygous 22q11.2 deletions and is characterized by malformations attributed to abnormal development of the...

Full spectrum of malformations in velo-cardio-facial syndrome/DiGeorge syndrome mouse models by altering Tbx1 dosage (2004)

Liao, Jun, Kochilas, Lazaros, Nowotschin, Sonja, Arnold, Jelena S., Aggarwal, Vimla S., Epstein, Jonathan A., ...

Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS) is associated with de novo hemizygous 22q11.2 deletions and is characterized by malformations attributed to abnormal development of the...

Full spectrum of malformations in velo-cardio-facial syndrome/DiGeorge syndrome mouse models by altering Tbx1 dosage (2004)

Liao, Jun, Kochilas, Lazaros, Nowotschin, Sonja, Arnold, Jelena S., Aggarwal, Vimla S., Epstein, Jonathan A., ...

Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS) is associated with de novo hemizygous 22q11.2 deletions and is characterized by malformations attributed to abnormal development of the...

Frequent translocations occur between low copy repeats on chromosome 22q11.2 (LCR22s) and telomeric bands of partner chromosomes (2003)

Spiteri, Elizabeth, Babcock, Melanie, Kashork, Catherine D., Wakui, Keiko, Gogineni, Swarna, Lewis, Debbie A., ...

The chromosome 22q11.2 region is susceptible to rearrangements, mediated by low copy repeats (LCR22s). Deletions and duplications are mediated by homologous recombination events between LCR22s. The...

Shuffling of Genes Within Low-Copy Repeats on 22q11 (LCR22) by Alu-Mediated Recombination Events During Evolution (2003)

Babcock, Melanie, Pavlicek, Adam, Spiteri, Elizabeth, Kashork, Catherine D., Ioshikhes, Ilya, Shaffer, Lisa G., ...

Low-copy repeats, or segmental duplications, are highly dynamic regions in the genome. The low-copy repeats on chromosome 22q11.2 (LCR22) are a complex mosaic of genes and pseudogenes formed by...

Mice overexpressing genes from the 22q11 region deleted in velo-cardio-facial syndrome/DiGeorge syndrome have middle and inner ear defects (2001)

Funke, Birgit, Epstein, Jonathan A., Kochilas, Lazaros K., Lu, Min Min, Pandita, Raj K., Liao, Jun, ...

Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS) is a congenital anomaly disorder associated with hemizygous 22q11 deletions. We previously showed that bacterial artificial chromosome...

A common molecular basis for rearrangement disorders on chromosome 22q11 (1999)

Edelmann, Lisa, Pandita, Raj K., Spiteri, Elizabeth, Funke, Birgit, Goldberg, Rosalie, Palanisamy, Nallasivam, ...

The chromosome 22q11 region is susceptible to rearrangements that are associated with congenital anomaly disorders and malignant tumors. Three congenital anomaly disorders, cat-eye syndrome, der(22)...

Two Functional Copies of the DGCR6 Gene Are Present on Human Chromosome 22q11 Due to a Duplication of an Ancestral Locus

Edelmann, Lisa, Stankiewicz, Pavel, Spiteri, Elizabeth, Pandita, Raj K., Shaffer, Lisa, Lupski, James, ...

The DGCR6 (DiGeorge critical region) gene encodes a putative protein with sequence similarity to gonadal (gdl), a Drosophila melanogaster gene of unknown function. We mapped the DGCR6 gene to...

Shuffling of Genes Within Low-Copy Repeats on 22q11 (LCR22) by Alu-Mediated Recombination Events During Evolution

Babcock, Melanie, Pavlicek, Adam, Spiteri, Elizabeth, Kashork, Catherine D., Ioshikhes, Ilya, Shaffer, Lisa G., ...

Low-copy repeats, or segmental duplications, are highly dynamic regions in the genome. The low-copy repeats on chromosome 22q11.2 (LCR22) are a complex mosaic of genes and pseudogenes formed by...

Traffic of genetic information between segmental duplications flanking the typical 22q11.2 deletion in velo-cardio-facial syndrome/DiGeorge syndrome

Pavlicek, Adam, House, Reniqua, Gentles, Andrew J., Jurka, Jerzy, Morrow, Bernice E.

Velo-cardio-facial syndrome/DiGeorge syndrome results from unequal crossing-over events between two 240-kb low-copy repeats termed LCR22 (LCR22-2 and LCR22-4) on Chromosome 22q11.2, comprised of...

Two Functional Copies of the DGCR6 Gene Are Present on Human Chromosome 22q11 Due to a Duplication of an Ancestral Locus

Edelmann, Lisa, Stankiewicz, Pavel, Spiteri, Elizabeth, Pandita, Raj K., Shaffer, Lisa, Lupski, James, ...

The DGCR6 (DiGeorge critical region) gene encodes a putative protein with sequence similarity to gonadal (gdl), a Drosophila melanogaster gene of unknown function. We mapped the DGCR6 gene to...

Shuffling of Genes Within Low-Copy Repeats on 22q11 (LCR22) by Alu-Mediated Recombination Events During Evolution

Babcock, Melanie, Pavlicek, Adam, Spiteri, Elizabeth, Kashork, Catherine D., Ioshikhes, Ilya, Shaffer, Lisa G., ...

Low-copy repeats, or segmental duplications, are highly dynamic regions in the genome. The low-copy repeats on chromosome 22q11.2 (LCR22) are a complex mosaic of genes and pseudogenes formed by...

Genomic Disorders on 22q11

McDermid, Heather E., Morrow, Bernice E.

The 22q11 region is involved in chromosomal rearrangements that lead to altered gene dosage, resulting in genomic disorders that are characterized by mental retardation and/or congenital...

Microduplication and Triplication of 22q11.2: A Highly Variable Syndrome

Yobb, Twila M., Somerville, Martin J., Willatt, Lionel, Firth, Helen V., Harrison, Karen, MacKenzie, Jennifer, ...

22q11.2 microduplications of a 3-Mb region surrounded by low-copy repeats should be, theoretically, as frequent as the deletions of this region; however, few microduplications have been reported. We...

Traffic of genetic information between segmental duplications flanking the typical 22q11.2 deletion in velo-cardio-facial syndrome/DiGeorge syndrome

Pavlicek, Adam, House, Reniqua, Gentles, Andrew J., Jurka, Jerzy, Morrow, Bernice E.

Velo-cardio-facial syndrome/DiGeorge syndrome results from unequal crossing-over events between two 240-kb low-copy repeats termed LCR22 (LCR22-2 and LCR22-4) on Chromosome 22q11.2, comprised of...

AT-rich repeats associated with chromosome 22q11.2 rearrangement disorders shape human genome architecture on Yq12

Babcock, Melanie, Yatsenko, Svetlana, Stankiewicz, Pawel, Lupski, James R., Morrow, Bernice E.

Low copy repeats (LCRs; segmental duplications) constitute ∼5% of the sequenced human genome. Nonallelic homologous recombination events between LCRs during meiosis can lead to chromosomal...

Cooperative Function of Tbx1 and Brn4 in the Periotic Mesenchyme is Necessary for Cochlea Formation

Braunstein, Evan M., Crenshaw III, E. Bryan, Morrow, Bernice E., Adams, Joe C.

The T-box transcription factor TBX1 has been identified as the major gene responsible for the etiology of velocardiofacial syndrome/DiGeorge syndrome (VCFS/DGS). Conductive hearing loss occurs in a...