Automated Author Profile

Preobrazhenskaya, E.V.

Current S-Index

0.4

Sum of Dataset Indices for all datasets

Average Dataset Index per Dataset

0.2

Average Dataset Index per dataset

Total Datasets

2

Total datasets for this author

Average FAIR Score

13.5%

Average FAIR Score per dataset

Total Citations

0

Total citations to the author's datasets

Total Mentions

0

Total mentions of the author's datasets

S-Index Interpretation

S-Index Over Time

Cumulative Citations Over Time

Cumulative Mentions Over Time

Datasets

Supplementary Material for: Exome Sequencing of a Family with Bardet-Biedl Syndrome Identifies the Common Russian Mutation c.1967_1968delTAinsC in <b><i>BBS7</i></b>

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive ciliopathy characterized by obesity, postaxial polydactyly, retinitis pigmentosa, mental retardation, and kidney abnormalities. At least 19 genes have been shown to be associated with BBS, and therefore, genetic testing is highly complicated. We used an Illumina MiSeq platform for whole exome sequencing analysis of a family with strong clinical features of BBS. A homozygous c.1967_1968delTAinsC (p.Leu656fsX673; RefSeq NM_176824.2) mutation in BBS7 was identified in both affected children, while their healthy sibling and the non-consanguineous parents were heterozygous for this allele. Genotyping of 2,832 DNA samples obtained from Russian blood donors revealed 2 additional heterozygous subjects (0.07%) with the c.1967_1968delTAinsC mutation. These findings may facilitate the genetic diagnosis for Slavic BBS patients.

Authors

  • Suspitsin, E.N. ;
  • Sokolenko, A.P. ;
  • Lyazina, L.V. ;
  • Preobrazhenskaya, E.V. ;
  • Lepenchuk, A.Y. ;
  • Imyanitov, E.N.
0 Citations0 Mentions13% FAIR0.1 Dataset Index
10.6084/m9.figshare.5127511January 2015

Supplementary Material for: Exome Sequencing of a Family with Bardet-Biedl Syndrome Identifies the Common Russian Mutation c.1967_1968delTAinsC in <b><i>BBS7</i></b>

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive ciliopathy characterized by obesity, postaxial polydactyly, retinitis pigmentosa, mental retardation, and kidney abnormalities. At least 19 genes have been shown to be associated with BBS, and therefore, genetic testing is highly complicated. We used an Illumina MiSeq platform for whole exome sequencing analysis of a family with strong clinical features of BBS. A homozygous c.1967_1968delTAinsC (p.Leu656fsX673; RefSeq NM_176824.2) mutation in BBS7 was identified in both affected children, while their healthy sibling and the non-consanguineous parents were heterozygous for this allele. Genotyping of 2,832 DNA samples obtained from Russian blood donors revealed 2 additional heterozygous subjects (0.07%) with the c.1967_1968delTAinsC mutation. These findings may facilitate the genetic diagnosis for Slavic BBS patients.

Authors

  • Suspitsin, E.N. ;
  • Sokolenko, A.P. ;
  • Lyazina, L.V. ;
  • Preobrazhenskaya, E.V. ;
  • Lepenchuk, A.Y. ;
  • Imyanitov, E.N.
0 Citations0 Mentions13% FAIR0.3 Dataset Index
10.6084/m9.figshare.5127511.v1January 2015