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Brian A. Kudlow
University of Washington
LaminProgeriaLMNAGeneticsBiology
11Publications
10H-index
1,000Citations
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Publications 11
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#1Brian A. Kudlow (UW: University of Washington)H-Index: 10
#2Monique N. Stanfel (UW: University of Washington)H-Index: 2
Last. Brian K. Kennedy (UW: University of Washington)H-Index: 66
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Hutchinson-Gilford progeria syndrome (HGPS) is a rare, debilitating disease with early mortality and rapid onset of aging-associated pathologies. It is linked to mutations in LMNA, which encodes A-type nuclear lamins. The most frequent HGPS-associated LMNA mutation results in a protein, termed progerin, with an internal 50 amino acid deletion and, unlike normal A-type lamins, stable farnesylation. The cellular consequences of progerin expression underlying the HGPS phenotype remain poorly unders...
82 CitationsSource
#1Torn Schmidlin (UW: University of Washington)H-Index: 1
#2Matt Kaeberlein (UW: University of Washington)H-Index: 55
Last. Brian K. Kennedy (UW: University of Washington)H-Index: 66
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Using the Saccharomyces cerevisiae MATa/MATα ORF deletion collection, homozygous deletion strains were identified that undergo mating with MATa or MATα haploids. Seven homozygous deletions were identified that confer enhanced mating. Three of these, lacking CTF8, CTF18, and DCC1, mate at a low frequency with either MATa or MATα haploids. The products of these genes form a complex involved in sister chromatid cohesion. Each of these strains also exhibits increased chromosome loss rates, and matin...
12 CitationsSource
#1Brian A. Kudlow (UW: University of Washington)H-Index: 10
#2Brian K. Kennedy (UW: University of Washington)H-Index: 66
Last. Raymond J. Monnat (UW: University of Washington)H-Index: 39
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Recent data on the genetic and molecular basis of progeroid syndromes have shed light onto the nuclear metabolic defects that might accelerate ageing. What are the mechanistic features of progeroid syndromes? And how can these findings help us understand normal ageing?
189 CitationsSource
#1Brian A. Kudlow (UW: University of Washington)H-Index: 10
#2Brian K. Kennedy (UW: University of Washington)H-Index: 66
The relationship between progerias — diseases that resemble premature aging — and the normal aging process has been a source of debate in the aging research community. A recent study finds that LMNA , a gene targeted for mutation in Hutchinson Gilford Progeria Syndrome, may control the onset of aging-associated decline in normal fibroblasts.
15 CitationsSource
A-type lamins are intermediate filament proteins that have been linked to the organization and maintenance of nuclear structure. In most differentiated tissues, A-type lamins (predominantly lamins A and C), along with lamin B, comprise the meshwork underlying the inner nuclear membrane known as the nuclear lamina (63). Unlike lamin C, lamin A contains a carboxy-terminal CaaX motif and must undergo a series of posttranslational modifications to form the mature lamin A (77). In particular, the cys...
69 CitationsSource
#1Richard L. Frock (UW: University of Washington)H-Index: 14
#2Brian A. Kudlow (UW: University of Washington)H-Index: 10
Last. Brian K. Kennedy (UW: University of Washington)H-Index: 66
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Mutations within LMNA, encoding A-type nuclear lamins, are associated with multiple tissue-specific diseases, including Emery-Dreifuss (EDMD2/3) and Limb-Girdle muscular dystrophy (LGMD1B). X-linked EDMD results from mutations in emerin, a lamin A-associated protein. The mechanisms through which these mutations cause muscular dystrophy are not understood. Here we show that most, but not all, cultured muscle cells from lamin A/C knockout mice exhibit impaired differentiation kinetics and reduced ...
187 CitationsSource
#1Brian A. KudlowH-Index: 10
Last. Brian K. KennedyH-Index: 66
view all 3 authors...
Objectives: To determine the importance of lamin A/C for fat cell differentiation in vitro and for the anti-adipogenic activity of HIV protease inhibitors such as indinavir. Methods: Lipodystrophy-associated and processing-defective mutants of lamin A were stably expressed at high levels in 3T3-L1 pre-adipocytes. Additionally, 3T3-L1 pre-adipocytes with stable reduction of lamin A/C or emerin were derived. The cells were differentiated for 8 days into mature adipocytes in the presence or absence...
26 CitationsSource
#1Erica D. Smith (UW: University of Washington)H-Index: 11
#2Brian A. Kudlow (UW: University of Washington)H-Index: 10
Last. Brian K. Kennedy (UW: University of Washington)H-Index: 66
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Nuclear lamins were identified as core nuclear matrix constituents over 20 years ago. They have been ascribed structural roles such as maintaining nuclear integrity and assisting in nuclear envelope formation after mitosis, and have also been linked to nuclear activities including DNA replication and transcription. Recently, A-type lamin mutations have been linked to a variety of rare human diseases including muscular dystrophy, lipodystrophy, cardiomyopathy, neuropathy and progeroid syndromes (...
43 CitationsSource
#1David A. BarbieH-Index: 33
#2Brian A. KudlowH-Index: 10
Last. Brian K. KennedyH-Index: 66
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In primary mammalian cells, DNA replication initiates in a small number of perinucleolar, lamin A/C-associated foci. During S-phase progression in proliferating cells, replication foci distribute to hundreds of sites throughout the nucleus. In contrast, we find that the limited perinucleolar replication sites persist throughout S phase as cells prepare to exit the cell cycle in response to contact inhibition, serum starvation, or replicative senescence. Proteins known to be involved in DNA synth...
32 CitationsSource
#1Lishan Chen (UW: University of Washington)H-Index: 4
#2Lin Lee (UW: University of Washington)H-Index: 8
Last. Junko Oshima (UW: University of Washington)H-Index: 45
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Summary Background Werner's syndrome is a progeroid syndrome caused by mutations at the WRN helicase locus. Some features of this disorder are also present in laminopathies caused by mutant LMNA encoding nuclear lamin A/C. Because of this similarity, we sequenced LMNA in individuals with atypical Werner's syndrome (wild-type WRN ). Methods Of 129 index patients referred to our international registry for molecular diagnosis of Werner's syndrome, 26 (20%) had wildtype WRN coding regions and were c...
344 CitationsSource
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