Lamin B1

Last updated
LMNB1
Protein LMNB1 PDB 2KPW.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases LMNB1 , ADLD, LMN, LMN2, LMNB, lamin B1, MCPH26
External IDs OMIM: 150340 MGI: 96795 HomoloGene: 55912 GeneCards: LMNB1
Orthologs
SpeciesHumanMouse
Entrez

4001

16906

Ensembl

ENSG00000113368

ENSMUSG00000024590

UniProt

P20700

P14733

RefSeq (mRNA)

NM_001198557
NM_005573

NM_010721

RefSeq (protein)

NP_001185486
NP_005564

NP_034851

Location (UCSC) Chr 5: 126.78 – 126.84 Mb Chr 18: 56.84 – 56.89 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Lamin-B1 is a protein that in humans is encoded by the LMNB1 gene. [5] [6] [7]

Contents

The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure, and gene expression. Vertebrate lamins consist of two types, A and B. This gene encodes one of the two B type proteins, B1. [7] Lamin B, along with heterochromatin, is anchored to the inner surface of the nuclear membrane by the lamin B receptor.

Interactions

LMNB1 has been shown to interact with Thymopoietin. [8] [9] When double-strand breaks are induced in DNA by ionizing radiation, lamin B1 promotes repair of the breaks, as well as cell survival, by maintaining the level of the RAD51 protein that is employed in homologous recombinational repair. [10]

Pathology

Mutations affecting the LMNB1 gene cause autosomal dominant adult-onset demyelinating leukodystrophy.

See also

Related Research Articles

<span class="mw-page-title-main">Nuclear lamina</span>

The nuclear lamina is a dense fibrillar network inside the nucleus of eukaryote cells. It is composed of intermediate filaments and membrane associated proteins. Besides providing mechanical support, the nuclear lamina regulates important cellular events such as DNA replication and cell division. Additionally, it participates in chromatin organization and it anchors the nuclear pore complexes embedded in the nuclear envelope.

<span class="mw-page-title-main">Thymopoietin</span>

Lamina-associated polypeptide 2 (LAP2), isoforms beta/gamma is a protein that in humans is encoded by the TMPO gene. LAP2 is an inner nuclear membrane (INM) protein.

<span class="mw-page-title-main">Laminopathy</span> Medical condition

Laminopathies are a group of rare genetic disorders caused by mutations in genes encoding proteins of the nuclear lamina. They are included in the more generic term nuclear envelopathies that was coined in 2000 for diseases associated with defects of the nuclear envelope. Since the first reports of laminopathies in the late 1990s, increased research efforts have started to uncover the vital role of nuclear envelope proteins in cell and tissue integrity in animals.

<span class="mw-page-title-main">Prelamin-A/C</span> Filament protein

Prelamin-A/C, or lamin A/C is a protein that in humans is encoded by the LMNA gene. Lamin A/C belongs to the lamin family of proteins.

<span class="mw-page-title-main">Lamin B receptor</span> Protein-coding gene in the species Homo sapiens

Lamin-B receptor is a protein, and in humans, it is encoded by the LBR gene.

<span class="mw-page-title-main">LEM domain-containing protein 3</span>

LEM domain-containing protein 3 (LEMD3), also known as MAN1, is an integral protein in the inner nuclear membrane (INM) of the nuclear envelope. It is encoded by the LEMD3 gene and was first identified after it was isolated from the serum of a patient with a collagen vascular disease.

<span class="mw-page-title-main">CBX3</span> Protein-coding gene in the species Homo sapiens

Chromobox protein homolog 3 is a protein that is encoded by the CBX3 gene in humans.

<span class="mw-page-title-main">Nucleoporin 210kDa</span> Protein-coding gene in the species Homo sapiens

Nuclear pore glycoprotein-210 (gp210) is an essential trafficking regulator in the eukaryotic nuclear pore complex. Gp-210 anchors the pore complex to the nuclear membrane. and protein tagging reveals its primarily located on the luminal side of double layer membrane at the pore. A single polypeptide motif of gp210 is responsible for sorting to nuclear membrane, and indicate the carboxyl tail of the protein is oriented toward the cytoplasmic side of the membrane.

<span class="mw-page-title-main">Cyclin B1</span> Protein-coding gene in the species Homo sapiens

G2/mitotic-specific cyclin-B1 is a protein that in humans is encoded by the CCNB1 gene.

<span class="mw-page-title-main">CDC25B</span> Protein-coding gene in the species Homo sapiens

M-phase inducer phosphatase 2 is an enzyme that in humans is encoded by the CDC25B gene.

<span class="mw-page-title-main">Wee1-like protein kinase</span> Protein-coding gene in the species Homo sapiens

WEE1 homolog , also known as WEE1, is a protein which in humans is encoded by the WEE1 gene.

<span class="mw-page-title-main">Nuclear mitotic apparatus protein 1</span> Protein-coding gene in the species Homo sapiens

Nuclear mitotic apparatus protein 1 is a protein that in humans is encoded by the NUMA1 gene.

<span class="mw-page-title-main">MMP16</span> Protein-coding gene in the species Homo sapiens

Matrix metalloproteinase-16 is an enzyme that in humans is encoded by the MMP16 gene.

<span class="mw-page-title-main">AKAP1</span> Protein-coding gene in the species Homo sapiens

A kinase anchor protein 1, mitochondrial is an enzyme that in humans is encoded by the AKAP1 gene.

<span class="mw-page-title-main">AKAP8</span> Protein-coding gene in the species Homo sapiens

A-kinase anchor protein 8 is an enzyme that, in humans, is encoded by the AKAP8 gene.

<span class="mw-page-title-main">PLK2</span> Protein-coding gene in the species Homo sapiens

Serine/threonine-protein kinase PLK2 is an enzyme that in humans is encoded by the PLK2 gene.

<span class="mw-page-title-main">UNC84A</span> Protein-coding gene in the species Homo sapiens

Protein unc-84 homolog A is a protein that in humans is encoded by the UNC84A gene.

<span class="mw-page-title-main">TOR1AIP1</span> Protein-coding gene in the species Homo sapiens

Torsin-1A-interacting protein 1 is a protein that in humans is encoded by the TOR1AIP1 gene. More commonly known as lamina associated polypeptide 1 (LAP1), it is a type II integral membrane protein that resides in the inner nuclear membrane. The luminal domain of LAP1 interacts with Torsin A and is necessary for the ATPase activity of Torsin A. LAP1 plays a critical role in skeletal and heart muscle. Mutations in TOR1AIP1 have been linked to muscular dystrophy and cardiomyopathy. It's deletion from mouse hepatocytes leads to defected very-low density lipoprotein secretion and causes non-alcoholic fatty liver disease and non-alcoholic steatohepatitis

<span class="mw-page-title-main">Nuclear prelamin A recognition factor</span> Protein-coding gene in the species Homo sapiens

Nuclear prelamin A recognition factor, also known as NARF, is a protein which in humans is encoded by the NARF gene.

<span class="mw-page-title-main">Inner nuclear membrane protein</span> Protein embedded in inner membrane of nuclear envelope

Inner nuclear membrane proteins are membrane proteins that are embedded in or associated with the inner membrane of the nuclear envelope. There are about 60 INM proteins, most of which are poorly characterized with respect to structure and function. Among the few well-characterized INM proteins are lamin B receptor (LBR), lamina-associated polypeptide 1 (LAP1), lamina-associated polypeptide-2 (LAP2), emerin and MAN1.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000113368 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000024590 - Ensembl, May 2017
  3. ↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ↑ Lin F, Worman HJ (Nov 1995). "Structural organization of the human gene (LMNB1) encoding nuclear lamin B1". Genomics. 27 (2): 230–6. doi: 10.1006/geno.1995.1036 . PMID   7557986.
  6. ↑ Wydner KL, McNeil JA, Lin F, Worman HJ, Lawrence JB (Feb 1997). "Chromosomal assignment of human nuclear envelope protein genes LMNA, LMNB1, and LBR by fluorescence in situ hybridization". Genomics. 32 (3): 474–8. doi: 10.1006/geno.1996.0146 . PMID   8838815.
  7. 1 2 "Entrez Gene: LMNB1 lamin B1".
  8. ↑ Furukawa K, Kondo T (Feb 1998). "Identification of the lamina-associated-polypeptide-2-binding domain of B-type lamin". Eur. J. Biochem. GERMANY. 251 (3): 729–33. doi: 10.1046/j.1432-1327.1998.2510729.x . ISSN   0014-2956. PMID   9490046.
  9. ↑ Foisner R, Gerace L (Jul 1993). "Integral membrane proteins of the nuclear envelope interact with lamins and chromosomes, and binding is modulated by mitotic phosphorylation". Cell. UNITED STATES. 73 (7): 1267–79. doi:10.1016/0092-8674(93)90355-T. ISSN   0092-8674. PMID   8324822. S2CID   10641633.
  10. ↑ Liu NA, Sun J, Kono K, Horikoshi Y, Ikura T, Tong X, Haraguchi T, Tashiro S. Regulation of homologous recombinational repair by lamin B1 in radiation-induced DNA damage. FASEB J. 2015 Jun;29(6):2514-25. doi: 10.1096/fj.14-265546. Epub 2015 Mar 2. PMID: 25733566

Further reading


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