Anti-ANTXR2 Antibody (CAB6526)
- SKU:
- CAB6526
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-ANTXR2 Antibody |
抗体コード: | CAB6526 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 339-488 of human ANTXR2 (NP_477520.2). |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | Mouse eye, Rat lung |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 339-488 of human ANTXR2 (NP_477520.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | WFWP LCCK VVIK DPPP PPAP APKE EEEE PLPT KKWP TVDA SYYG GRGV GGIK RMEV RWGD KGST EEGA RLEK AKNA VVKI PEET EEPI RPRP PRPK PTHQ PPQT KWYT PIKG RLDA LWAL LRRQ YDRV SLMR PQEG DEGR CINF SRVP SQ |
遺伝子ID: | 118429 |
Uniprot: | P58335 |
セルラーロケーション: | Cell membrane, Endoplasmic reticulum membrane, Secreted, Single-pass type I membrane protein, Single-pass type I membrane protein |
計算された分子量: | 34kDa/42kDa/53kDa |
観察された分子量: | 54kDa |
同義語: | ANTXR2, CMG-2, CMG2, HFS, ISH, JHF |
バックグラウンド: | This gene encodes a receptor for anthrax toxin. The protein binds to collagen IV and laminin, suggesting that it may be involved in extracellular matrix adhesion. Mutations in this gene cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Multiple transcript variants encoding different isoforms have been found for this gene. |
UniProt Protein Function: | ANTXR2: Necessary for cellular interactions with laminin and the extracellular matrix. Defects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH). This autosomal recessive syndrome is similar to JHF, but has an earlier onset and a more severe course. Symptoms appear at birth or within the first months of life, with painful, swollen joint contractures, osteopenia, osteoporosis and livid red hyperpigmentation over bony prominences. Patients develop multiple subcutaneous skin tumors and gingival hypertrophy. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to death within the first 2 years of life. Surviving children may suffer from severely reduced mobility due to joint contractures. Defects in ANTXR2 are the cause of juvenile hyaline fibromatosis (JHF). JHF is an autosomal recessive syndrome that is similar to ISH but takes a milder course. It is characterized by hyaline deposition in the dermis, multiple subcutaneous skin tumors and gingival hypertrophy, followed by progressive joint contractions, osteopenia and osteoporosis that may lead to a severe limitation of mobility. Belongs to the ATR family. 4 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Receptor, misc. Chromosomal Location of Human Ortholog: 4q21.21 Cellular Component: endoplasmic reticulum membrane; extracellular region; plasma membrane; integral to membrane; endosome membrane; external side of plasma membrane Molecular Function:protein binding; metal ion binding; receptor activity Biological Process: reproductive process; pathogenesis Disease: Hyaline Fibromatosis Syndrome |
NCBI Summary: | This gene encodes a receptor for anthrax toxin. The protein binds to collagen IV and laminin, suggesting that it may be involved in extracellular matrix adhesion. Mutations in this gene cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009] |
UniProt Code: | P58335 |
NCBI GenInfo Identifier: | 306526289 |
NCBI Gene ID: | 118429 |
NCBI Accession: | P58335.5 |
UniProt Secondary Accession: | P58335,Q4W5H6, Q59E98, Q5JPE9, Q86UI1, Q8N4J8, Q8NB13 Q96NC7, |
UniProt Related Accession: | P58335 |
Molecular Weight: | 53,493 Da |
NCBI Full Name: | Anthrax toxin receptor 2 |
NCBI Synonym Full Names: | anthrax toxin receptor 2 |
NCBI Official Symbol: | ANTXR2 |
NCBI Official Synonym Symbols: | HFS; ISH; JHF; CMG2; CMG-2 |
NCBI Protein Information: | anthrax toxin receptor 2; capillary morphogenesis protein 2; capillary morphogenesis gene 2 protein |
UniProt Protein Name: | Anthrax toxin receptor 2 |
UniProt Synonym Protein Names: | Capillary morphogenesis gene 2 protein; CMG-2 |
Protein Family: | Anthrax toxin receptor |
UniProt Gene Name: | ANTXR2 |
UniProt Entry Name: | ANTR2_HUMAN |
View AllClose