LBR Antibody (PACO16606)
- SKU:
- PACO16606
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | LBR Antibody (PACO16606) |
抗体コード: | PACO16606 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:2000-1:5000, WB:1:500-1:2000, IHC:1:50-1:200 |
反応性: | Human |
免疫原: | Fusion protein of human LBR |
憲法: | Liquid |
ストレージバッファ: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
精製方法: | Antigen affinity purification |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane: Human fetal brain tissue, Primary antibody: PACO16606(LBR Antibody) at dilution 1/300, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 3 minutes. |
![]() | The image on the left is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using PACO16606(LBR Antibody) at dilution 1/50, on the right is treated with fusion protein. (Original magnification: x200). |
バックグラウンド: | The protein encoded by this gene belongs to the ERG4/ERG24 family. It localized in the nuclear envelope inner membrane and anchors the lamina and the heterochromatin to the membrane. It may mediate interaction between chromatin and lamin B. Mutations of this gene has been associated with autosomal recessive HEM/Greenberg skeletal dysplasia. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been identified. |
シノニム: | lamin B receptor |
UniProt Protein Function: | LBR: Anchors the lamina and the heterochromatin to the inner nuclear membrane. Defects in LBR are a cause of Pelger-Huet anomaly (PHA). PHA is an autosomal dominant inherited abnormality of neutrophils, characterized by reduced nuclear segmentation and an apparently looser chromatin structure. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin. Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy, and skeletal abnormalities. Defects in LBR are the cause of hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM); also known as Greenberg skeletal dysplasia. HEM is a rare autosomal recessive chondrodystrophy characterized by early in utero lethality and, therefore, considered to be nonviable. Affected fetuses typically present with fetal hydrops, short- limbed dwarfism, and a marked disorganization of chondro-osseous calcification and may present with polydactyly and additional nonskeletal malformations. Defects in LBR may be a cause of Reynolds syndrome (REYNS). It is a syndrome specifically associating limited cutaneous systemic sclerosis and primary biliray cirrhosis. It is characterized by liver disease, telangiectasia, abrupt onset of digital paleness or cyanosis in response to cold exposure or stress (Raynaud phenomenon), and variable features of scleroderma. The liver disease is characterized by pruritis, jaundice, hepatomegaly, increased serum alkaline phosphatase and positive serum mitochondrial autoantibodies, all consistent with primary biliary cirrhosis. Belongs to the ERG4/ERG24 family. |
UniProt Protein Details: | Protein type:Membrane protein, multi-pass; Membrane protein, integral; DNA-binding Chromosomal Location of Human Ortholog: 1q42.1 Cellular Component: nuclear membrane; membrane; integral to membrane; integral to nuclear inner membrane; nuclear envelope Molecular Function:protein binding; DNA binding; oxidoreductase activity, acting on the CH-CH group of donors, NAD or NADP as acceptor; lamin binding Biological Process: cholesterol biosynthetic process Disease: Pelger-huet Anomaly; Greenberg Dysplasia; Reynolds Syndrome |
NCBI Summary: | The protein encoded by this gene belongs to the ERG4/ERG24 family. It localized in the nuclear envelope inner membrane and anchors the lamina and the heterochromatin to the membrane. It may mediate interaction between chromatin and lamin B. Mutations of this gene has been associated with autosomal recessive HEM/Greenberg skeletal dysplasia. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q14739 |
NCBI GenInfo Identifier: | 20141468 |
NCBI Gene ID: | 3930 |
NCBI Accession: | Q14739.2 |
UniProt Secondary Accession: | Q14739,Q14740, Q53GU7, Q59FE6, B2R5P3, |
UniProt Related Accession: | Q14739 |
Molecular Weight: | 615 |
NCBI Full Name: | Lamin-B receptor |
NCBI Synonym Full Names: | lamin B receptor |
NCBI Official Symbol: | LBR |
NCBI Official Synonym Symbols: | PHA; LMN2R; TDRD18; DHCR14B |
NCBI Protein Information: | lamin-B receptor; tudor domain containing 18; integral nuclear envelope inner membrane protein |
UniProt Protein Name: | Lamin-B receptor |
UniProt Synonym Protein Names: | Integral nuclear envelope inner membrane protein; LMN2R |
Protein Family: | Lamin-B receptor |
UniProt Gene Name: | LBR |
UniProt Entry Name: | LBR_HUMAN |