TMPRSS6, Polyclonal Antibody

IRIDA

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

The antibody detects endogenous levels of total TMPRSS6 protein.

Antigen affinity purification

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

0.9mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-TMPRSS6 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Total protein Ab

Immunohistochemistry (IHC)

Immunohistochemistry: 1: 20-100

The image on the left is immunohistochemistry of paraffin-embedded Human tonsil tissue using TMPRSS6 Antibody at dilution 1/35, on the right is treated with synthetic peptide. (Original magnification: x200)

91,333 Da

transmembrane protease, serine 6

transmembrane protease serine 6

Transmembrane protease serine 6

TMPS6_HUMAN

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]

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.

Protein type: EC 3.4.21.-; Membrane protein, integral; 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

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