TMPRSS6 Antibody, HRP conjugated (PACO37591)
- SKU:
- PACO37591
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | TMPRSS6 Antibody, HRP conjugated (PACO37591) |
抗体コード: | PACO37591 |
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 Transmembrane protease serine 6 protein (98-301AA) |
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: | HRP |
バックグラウンド: | Serine protease which hydrolyzes a range of proteins including type I collagen, fibronectin and fibrinogen. Can also activate urokinase-type plasminogen activator with low efficiency. May play a specialized role in matrix remodeling processes in liver. Through the cleavage of HFE2, a regulator of the expression of the iron absorption-regulating hormone hepicidin/HAMP, plays a role in iron homeostasis. |
シノニム: | Transmembrane protease serine 6 (EC 3.4.21.-) (Matriptase-2), TMPRSS6 |
UniProt Protein Function: | TMPRSS6: Serine protease which hydrolyzes a range of proteins including type I collagen, fibronectin and fibrinogen. Can also activate urokinase-type plasminogen activator with low efficiency. May play a specialized role in matrix remodeling processes in liver. Plays a role in the regulation of iron homeostasis, a process involving HAMP. Required to sense iron deficiency and suppress activation of the HAMP promoter. Defects in TMPRSS6 are the cause of iron-refractory iron deficiency anemia (IRIDA); also known as hypochromic microcytic anemia with defect in iron metabolism or hereditary iron-handling disorder or pseudo-iron-deficiency anemia. Key features include congenital hypochromic microcytic anemia, very low mean corpuscular erythrocyte volume, low transferrin saturation, abnormal iron absorption characterized by no hematologic improvement following treatment with oral iron, and abnormal iron utilization characterized by a sluggish, incomplete response to parenteral iron. Mutations leading to abrogation of TMPRSS6 activity are associated with IRIDA due to elevated levels of hepcidin, a negative regulator of plasma iron pool (PubMed:20232450). Belongs to the peptidase S1 family. 4 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Membrane protein, integral; EC 3.4.21.-; Protease Chromosomal Location of Human Ortholog: 22q12.3 Cellular Component: extracellular space; integral to membrane; plasma membrane Molecular Function:protein binding; serine-type endopeptidase activity Biological Process: cellular iron ion homeostasis; fibrinolysis; iron ion homeostasis; membrane protein proteolysis; negative regulation of transcription from RNA polymerase II promoter; negative regulation of transcription, DNA-dependent; positive regulation of transcription from RNA polymerase II promoter; proteolysis Disease: Iron-refractory Iron Deficiency Anemia |
NCBI Summary: | The protein encoded by this gene is a type II transmembrane serine proteinase that is found attached to the cell surface. The encoded protein may be involved in matrix remodeling processes in the liver. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014] |
UniProt Code: | Q8IU80 |
NCBI GenInfo Identifier: | 209572718 |
NCBI Gene ID: | 164656 |
NCBI Accession: | Q8IU80.3 |
UniProt Secondary Accession: | Q8IU80,Q5TI06, Q6ICC2, Q6UXD8, Q8IUE2, Q8IXV8, B0QYB4 B0QYB7, B0QYB8, |
UniProt Related Accession: | Q8IU80 |
Molecular Weight: | 91,333 Da |
NCBI Full Name: | Transmembrane protease serine 6 |
NCBI Synonym Full Names: | transmembrane protease, serine 6 |
NCBI Official Symbol: | TMPRSS6 |
NCBI Official Synonym Symbols: | IRIDA |
NCBI Protein Information: | transmembrane protease serine 6 |
UniProt Protein Name: | Transmembrane protease serine 6 |
UniProt Synonym Protein Names: | Matriptase-2 |
Protein Family: | Transmembrane protease serine |
UniProt Gene Name: | TMPRSS6 |
UniProt Entry Name: | TMPS6_HUMAN |