EFEMP2 Antibody, Biotin conjugated (PACO47385)
- SKU:
- PACO47385
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- Biotin
Frequently bought together:
Description
抗体名: | EFEMP2 Antibody, Biotin conjugated (PACO47385) |
抗体コード: | PACO47385 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human EGF-containing fibulin-like extracellular matrix protein 2 protein (26-165AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Biotin |
バックグラウンド: | basement membrane, extracellular exosome, extracellular region, extracellular vesicle, extracellular matrix structural constituent |
シノニム: | EGF-containing fibulin-like extracellular matrix protein 2 (Fibulin-4) (FIBL-4) (Protein UPH1), EFEMP2, FBLN4 |
UniProt Protein Function: | EFEMP2: Defects in EFEMP2 are the cause of cutis laxa, autosomal recessive, type 1B (ARCL1B). A connective tissue disorder characterized by characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. ARCL1B features include emphysema, lethal pulmonary artery occlusion, aortic aneurysm, cardiopulmonary insufficiency, birth fractures, arachnodactyly, and fragility of blood vessels. Belongs to the fibulin family. |
UniProt Protein Details: | Protein type:Secreted, signal peptide; Secreted Chromosomal Location of Human Ortholog: 11q13.1 Cellular Component: basement membrane; extracellular region Molecular Function:extracellular matrix structural constituent; protein binding Disease: Cutis Laxa, Autosomal Recessive, Type Ib |
NCBI Summary: | A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are cause of an autosomal recessive cutis laxa syndrome. Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Jan 2011] |
UniProt Code: | O95967 |
NCBI GenInfo Identifier: | 134047775 |
NCBI Gene ID: | 30008 |
NCBI Accession: | O95967.3 |
UniProt Secondary Accession: | O95967,O75967, A8K7R4, B3KM31, B3KQT1, |
UniProt Related Accession: | O95967 |
Molecular Weight: | 49,405 Da |
NCBI Full Name: | EGF-containing fibulin-like extracellular matrix protein 2 |
NCBI Synonym Full Names: | EGF containing fibulin like extracellular matrix protein 2 |
NCBI Official Symbol: | EFEMP2 |
NCBI Official Synonym Symbols: | MBP1; UPH1; FBLN4; ARCL1B |
NCBI Protein Information: | EGF-containing fibulin-like extracellular matrix protein 2 |
UniProt Protein Name: | EGF-containing fibulin-like extracellular matrix protein 2 |
UniProt Synonym Protein Names: | Fibulin-4; FIBL-4; Protein UPH1 |
Protein Family: | EGF-containing fibulin-like extracellular matrix protein |
UniProt Gene Name: | EFEMP2 |
UniProt Entry Name: | FBLN4_HUMAN |