TRPP
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TRPP (transient receptor potential polycystic) is a family of transient receptor potential ion channels which when mutated can cause
polycystic kidney disease Polycystic kidney disease (PKD or PCKD, also known as polycystic kidney syndrome) is a genetic disorder in which the renal tubules become structurally abnormal, resulting in the development and growth of multiple cysts within the kidney. These c ...
.


Subcategories

TRPP subunits can be divided into two subcategories depending on structural similarity.


Polycystic Kidney Disease 1 (PKD1)-Like Group

The first group, polycystic kidney disease 1 (PKD1)-like, contains polycystin-1 (Previously known as TRPP1), PKDREJ, PKD1L1, PKD1L2, and PKD1L3. Polycystin-1 contains numerous N-terminal adhesive domains that are important for cell-cell contact.Islam, M. S
Transient Receptor Potential Channels
Advances in Experimental Medicine and Biology. Vol 704.
This group of subunits also contain a large extracellular domain with numerous polycystin motifs. These motifs are of unknown function and are located between the S6 and S7 segments. The large intracellular C-terminal segment of TRPP1 seems to interact with TRPP2 to act as a signaling complex.


Polycystic Kidney Disease 2 (PKD2)-Like Group

This group of TRPP members (previously known as TRPP2-like) are: TRPP1 (previously known as TRPP2 or PKD2), TRPP2 (previously known as TRPP3 or PKDL2), and TRPP3 (previously known as TRPP5 or polycystin-L2).* Unlike the previous group, which contain 11 membrane-spanning segments, this group resemble other TRP channels, having 6 membrane-spanning segments with intracellular N- and C-termini. All of the members of this group contain a
coiled coil A coiled coil is a structural motif in proteins in which 2–7 alpha-helices are coiled together like the strands of a rope. (Dimers and trimers are the most common types.) Many coiled coil-type proteins are involved in important biological fu ...
region in their C-terminus involved in the interaction with the polycystin-1 group. TRPP1 and TRPP3 form constitutively active cation-selective ion channels that are permeable to calcium. TRPP2 has also been implicated in sour taste perception. Coupling of PKD1 and TRPP1 recruits TRPP1 to the membrane. Here, its activity is decreased and it suppresses the activation of
G proteins G proteins, also known as guanine nucleotide-binding proteins, are a family of proteins that act as molecular switches inside cells, and are involved in transmitting signals from a variety of stimuli outside a cell to its interior. Their ac ...
by PKD1.


Genes

* Group 1: polycystic kidney disease 1 (PKD1) like proteins **
PKD1 Polycystin 1 (often abbreviated to PC1) is a protein that in humans is encoded by the ''PKD1'' gene. Mutations of ''PKD1'' are associated with most cases of autosomal dominant polycystic kidney disease, a severe hereditary disorder of the kidneys ...
** ** ** ** * Group 2: polycystic kidney disease 2 (PKD2) like proteins ** TRPP1 ** TRPP2 ** TRPP3


See also

*
Polycystic kidney disease Polycystic kidney disease (PKD or PCKD, also known as polycystic kidney syndrome) is a genetic disorder in which the renal tubules become structurally abnormal, resulting in the development and growth of multiple cysts within the kidney. These c ...


References


External links

* * * {{Transient receptor potential channel modulators Membrane proteins Ion channels