Mandibuloacral dysplasia type A (MADA) is a rare laminopathy characterized by growth retardation, craniofacial anomalies, bone resorption at specific sites including clavicles, phalanges and mandibula, mottled cutaneous pigmentation, skin rigidity, partial lipodystrophy, and insulin resistance. The disorder is caused by recessive mutations of the LMNA gene encoding for A-type lamins. The molecular feature of MADA consists in the accumulation of the unprocessed lamin A precursor, which is detected at the nuclear rim and in intranuclear aggregates. Here, we report the characterization of prelamin A post-translational modifications in MADA cells that induce alterations in the chromatin arrangement and dislocation of nuclear envelope-associated...
The Publisher's final version can be found by following the DOI link.The nuclear envelope (NE) LINC ...
International audienceA-type lamins, the intermediate filament proteins participating in nuclear str...
Lamin A, a protein component of the nuclear lamina, is synthesized as a precursor named prelamin A, ...
Mandibuloacral dysplasia type A (MADA) is a rare laminopathy characterized by growth retardation, cr...
The Author(s) 2012. This article is published with open access at Springerlink.com Abstract Mandibul...
The autosomal recessive mandibuloacral dysplasia (MADA; OMIM #248370) is caused by a mutation in LMN...
The involvement of the nuclear envelope in the modulation of chromatin organization is strongly sugg...
Lamin A is a nuclear lamina constituent expressed in differentiated cells. Mutations in the LMNA gen...
Lamin A is a key component of the nuclear lamina produced through post-translational processing of i...
Lamin A is a key component of the nuclear lamina produced through post-translational processing of i...
Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder, characterized by postnatal gr...
Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder, characterized by postnatal gr...
The nuclear lamina contributes to the regulation of gene expression and to chromatin organization. M...
[[abstract]]The inner nuclear envelope protein Sun1 interacts with the nuclear lamina and participat...
Pre-lamin A undergoes subsequent steps of post-translational modification at its C-terminus, includi...
The Publisher's final version can be found by following the DOI link.The nuclear envelope (NE) LINC ...
International audienceA-type lamins, the intermediate filament proteins participating in nuclear str...
Lamin A, a protein component of the nuclear lamina, is synthesized as a precursor named prelamin A, ...
Mandibuloacral dysplasia type A (MADA) is a rare laminopathy characterized by growth retardation, cr...
The Author(s) 2012. This article is published with open access at Springerlink.com Abstract Mandibul...
The autosomal recessive mandibuloacral dysplasia (MADA; OMIM #248370) is caused by a mutation in LMN...
The involvement of the nuclear envelope in the modulation of chromatin organization is strongly sugg...
Lamin A is a nuclear lamina constituent expressed in differentiated cells. Mutations in the LMNA gen...
Lamin A is a key component of the nuclear lamina produced through post-translational processing of i...
Lamin A is a key component of the nuclear lamina produced through post-translational processing of i...
Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder, characterized by postnatal gr...
Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder, characterized by postnatal gr...
The nuclear lamina contributes to the regulation of gene expression and to chromatin organization. M...
[[abstract]]The inner nuclear envelope protein Sun1 interacts with the nuclear lamina and participat...
Pre-lamin A undergoes subsequent steps of post-translational modification at its C-terminus, includi...
The Publisher's final version can be found by following the DOI link.The nuclear envelope (NE) LINC ...
International audienceA-type lamins, the intermediate filament proteins participating in nuclear str...
Lamin A, a protein component of the nuclear lamina, is synthesized as a precursor named prelamin A, ...