Protein Name: Prelamin-A/C
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UniprotKB/SwissProt ID: LMNA_HUMAN (P02545)
Gene Name: LMNA
Synonyms: LMN1
Organism: Homo sapiens (Human).
Function: Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone. Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence.
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Note=Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin- A/C, which can then
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PDB :
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Protein disease:
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| Disease database |
Database Entry |
Disease information | | HPRD | 01035 | Cardiomyopathy, dilated, 1A | | HPRD | 01035 | Charcot-Marie-tooth disease, axonal, type 2B1 | | HPRD | 01035 | Emery-Dreifuss muscular dystrophy, autosomal dominant | | HPRD | 01035 | Emery-Dreifuss muscular dystrophy, autosomal recessive | | HPRD | 01035 | Hutchinson-Gilford progeria syndrome | | HPRD | 01035 | Hutchinson-Gilford progeria syndrome, atypical | | HPRD | 01035 | Lipoatrophy with diabetes, hepatic steatosis, hypertrophic cardiomyopathy, and leukomelanodermic papules | | HPRD | 01035 | Lipodystrophy, familial partial, type 2 | | HPRD | 01035 | Mandibuloacral dysplasia with type A lipodystrophy | | HPRD | 01035 | Muscular dystrophy, limb-girdle, type 1B | | HPRD | 01035 | Myopathy, early-onset, with progeroid features | | HPRD | 01035 | Restrictive dermopathy, lethal | | HPRD | 01035 | Werner syndrome, atypical |
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Network with metabolic pathway:
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Graphical Visualization of S-nitrosylation Sites:
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| Overview of Protein S-nitrosylation Sites with Functional and Structural Information |  |
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