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Emerin Antibody - Goat Anti-Emerin
Emerin is a serine rich nuclear membrane protein and a member of the nuclear lamina associated protein family. It mediates membrane anchorage to the cytoskeleton. Dreifuss-Emery muscular dystrophy is an X-linked inherited degenerative myopathy resulting from mutation in the emerin gene
£226.00
Lamin A/C mutant R453W Antibody ; Mouse Anti-Lamin A/C mutant R453W
Nuclear lamins are intermediate filament proteins that are the major structural component of the nuclear lamina on the inner surface of the nuclear envelope. Lamin A and Lamin C are splice variants of the Lamin A gene. Lamin A/C (CDCD1, LMN1, EMD2) expression is a hallmark of embryonic stem cell differentiation. In addition to adding structural integrity to the nucleus, lamins contribute to the makeup of the nuclear matrix. Lamins also help organize interphase chromatin through interactions with several chromatin proteins, including histones and Lap2, such that alteration in lamin organization (laminopathy) results in disruption of DNA replication, transcription and RNA processing. The R453W mutation is one of the most common causes of autosomal dominant Emery-Dreifuss muscular dystrophy (EDMD)
£226.00
Nup53 Antibody- Rabbit Anti-Nup53
Nup53 encodes a recently identified nuclear pore protein. Transport of macromolecules between the nucleus and the cytoplasm of eukaryotic cells occurs through the nuclear pore complex (NPC), a large macromolecular complex that spans the nuclear envelope. The yeast NPC shares several features with the vertebrate NPC, despite being smaller and less elaborate. Nup53p forms a complex with two other nucleoporins, Nup170p and Asm4p. The complex is found on both the nuclear and cytoplasmic faces of the NPC, and associates with Pse1p, a member of a protein family implicated in nuclear protein import, via direct interaction between Nup53p and Pse1p. Nup53p is structurally similar to Asm4p, and similar proteins sequences are found in several eukaryotes including human and other multicellular species. Nup53p is not essential; deletion of Nup53 and ASM4 causes slow growth and altered subcellular distribution of Pse1p. In a nup53 pse1 double mutant, NLS-containing proteins are mislocalized to the cytoplasm
£226.00
Progerin Antibody ; Mouse Anti-Progerin
Progerin is a 614 amino acid protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a point mutation (C1824T) in the LMNA gene that codes for lamin A and C. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a 50-amino acids internal deletion within the carboxyl-terminal domain of the protein. Approximately 80% of Hutchinson-Gilford progeria syndrome cases carry a single copy of the most common mutation, a silent point mutation, G608G (GGC > GGT), within exon 11 of LMNA gene
£226.00
