Cell Biology Antibodies 1
Anti-EFEMP2 Antibody (CAB10018)
- SKU:
- CAB10018
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
Antibody Name: | Anti-EFEMP2 Antibody |
Antibody SKU: | CAB10018 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Human |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 26-250 of human EFEMP2 (NP_058634.4). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:1000 |
Reactivity: | Human |
Positive Samples: | SH-SY5Y, HT-1080 |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 26-250 of human EFEMP2 (NP_058634.4). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | SPQD SEEP DSYT ECTD GYEW DPDS QHCR DVNE CLTI PEAC KGEM KCIN HYGG YLCL PRSA AVIN DLHG EGPP PPVP PAQH PNPC PPGY EPDD QDSC VDVD ECAQ ALHD CRPS QDCH NLPG SYQC TCPD GYRK IGPE CVDI DECR YRYC QHRC VNLP GSFR CQCE PGFQ LGPN NRSC VDVN ECDM GAPC EQRC FNSY GTFL CRCH QGYE LHRD GFSC SDID ECSY S |
Gene ID: | 30008 |
Uniprot: | O95967 |
Cellular Location: | Secreted |
Calculated MW: | 49kDa |
Observed MW: | 55kDa |
Synonyms: | EFEMP2, ARCL1B, FBLN4, MBP1, UPH1 |
Background: | 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. |
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 |
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