TRPM6
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TRPM6
TRPM6 is a transient receptor potential ion channel associated with hypomagnesemia with secondary hypocalcemia. See also * TRPM * Ruthenium red The inorganic dye ammoniated ruthenium oxychloride, also known as ruthenium red, is used in histology to stain aldehyde fixed mucopolysaccharides. Ruthenium red (RR) has also been used as a pharmacological tool to study specific cellular mechani ... References Further reading * * * * External links * Ion channels {{membrane-protein-stub ...
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Hypomagnesemia With Secondary Hypocalcemia
Hypomagnesemia with secondary hypocalcemia (HSH) is an autosomal recessive genetic disorder affecting intestinal magnesium absorption. Decreased intestinal magnesium reabsorption and the resulting decrease in serum magnesium levels is believed to cause lowered parathyroid hormone (PTH) output by the parathyroid gland. This results in decreased PTH and decreased serum calcium levels (hypocalcemia). This manifests in convulsions and spasms in early infancy which, if left untreated, can lead to mental retardation or death. HSH is caused by mutations in the TRPM6 gene. Pathophysiology HSH is caused by decreased intestinal magnesium reabsorption through TRPM6 channels. When expressed in cells, TRPM6 produces outwardly rectifying currents with the outward portion composed of Na+ ions and the inward portion of divalent cations (particularly magnesium and calcium). Inward flow of sodium ions is blocked by extracellular divalent cations. Increased intracellular magnesium concentrations als ...
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TRPM
TRPM is a family of transient receptor potential ion channels (''M'' standing for '' wikt:melastatin''). Functional TRPM channels are believed to form tetramers. The TRPM family consists of eight different channels, TRPM1–TRPM8. Unlike the TRPC and TRPV sub-families, TRPM subunits do not contain N-terminal ankyrin repeat motifs but, rather, contain entire functional proteins in their C-termini. TRPM6 and TRPM7, for example, contain functional α-kinase segments, which are a type of serine/threonine-specific protein kinase. Permeability and activation The relative permeability of calcium and magnesium varies widely among TRPM channels. *TRPM4 and TRPM5 are impermeable to calcium. *TRPM3, TRPM6 and TRPM7 are highly permeable to both calcium and magnesium. The mechanism of activation also varies greatly among TRPM channels. *TRPM2 is activated by ADP-ribose adenosine 5'-diphosphoribose and functions as a sensor of redox status in cells. *TRPM4 and TRPM5 are activated by intra ...
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Transient Receptor Potential
Transient receptor potential channels (TRP channels) are a group of ion channels located mostly on the plasma membrane of numerous animal cell types. Most of these are grouped into two broad groups: Group 1 includes TRPC ( "C" for canonical), TRPV ("V" for vanilloid), TRPVL ("VL" for vanilloid-like), TRPM ("M" for melastatin), TRPS ("S" for soromelastatin), TRPN ("N" for no mechanoreceptor potential C), and TRPA ("A" for ankyrin). Group 2 consists of TRPP ("P" for polycystic) and TRPML ("ML" for mucolipin). Other less-well categorized TRP channels exist, including yeast channels and a number of Group 1 and Group 2 channels present in non-animals. Many of these channels mediate a variety of sensations such as pain, temperature, different kinds of tastes, pressure, and vision. In the body, some TRP channels are thought to behave like microscopic thermometers and used in animals to sense hot or cold. Some TRP channels are activated by molecules found in spices like garlic (allicin) ...
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Ruthenium Red
The inorganic dye ammoniated ruthenium oxychloride, also known as ruthenium red, is used in histology to stain aldehyde fixed mucopolysaccharides. Ruthenium red (RR) has also been used as a pharmacological tool to study specific cellular mechanisms. Selectivity is a significant issue in such studies as RR is known to interact with many proteins. These include mammalian ion channels (CatSper1, TASK, RyR1, RyR2, RyR3, TRPM6, TRPM8, TRPV1, TRPV2, TRPV3, TRPV4, TRPV5, TRPV6, TRPA1, mCa1, mCa2, CALHM1) TRPP3, a plant ion channel, Ca2+-ATPase, mitochondrial Ca2+ uniporter, tubulin, myosin light-chain phosphatase, and Ca2+ binding proteins such as calmodulin. Ruthenium red displays nanomolar potency against several of its binding partners (e.g. TRPV4, ryanodine receptors,...). For example, it is a potent inhibitor of intracellular calcium release by ryanodine receptors (Kd ~20 nM). As a TRPA1 blocker, it assists in reducing the airway inflammation caused by pepper spray. RR has been ...
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Ion Channel
Ion channels are pore-forming membrane proteins that allow ions to pass through the channel pore. Their functions include establishing a resting membrane potential, shaping action potentials and other electrical signals by gating the flow of ions across the cell membrane, controlling the flow of ions across secretory and epithelial cells, and regulating cell volume. Ion channels are present in the membranes of all cells. Ion channels are one of the two classes of ionophoric proteins, the other being ion transporters. The study of ion channels often involves biophysics, electrophysiology, and pharmacology, while using techniques including voltage clamp, patch clamp, immunohistochemistry, X-ray crystallography, fluoroscopy, and RT-PCR. Their classification as molecules is referred to as channelomics. Basic features There are two distinctive features of ion channels that differentiate them from other types of ion transporter proteins: #The rate of ion transport through the ...
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