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Details on Person UniProt:Q4KL25 Lhfpl5

Class:IdReferenceGeneProduct:251949
_chainChangeLogchain:1-219 added on Fri February 6 2015
_displayNameUniProt:Q4KL25 Lhfpl5
_timestamp2024-08-09 18:53:09
chainchain:1-219
checksum9BFC49260AE9DD02
commentFUNCTION Auxiliary subunit of the mechanotransducer (MET) non-specific cation channel complex located at the tips of the shorter stereocilia of cochlear hair cells and that mediates sensory transduction in the auditory system (PubMed:15905332, PubMed:23217710, PubMed:36191207). The MET complex is composed of two dimeric pore-forming ion-conducting transmembrane TMC (TMC1 or TMC2) subunits, and aided by several auxiliary proteins including LHFPL5, TMIE, CIB2/3 and TOMT, and the tip-link PCDH15 (PubMed:23217710, PubMed:36191207). Functionally couples PCDH15 to the transduction channel (PubMed:23217710).SUBUNIT Forms the MET channel composed of TMC (TMC1 or TMC2), TMIE, TOMT, CIB (CIB2 or CIB3), LHPL5 and PCDH15 (PubMed:23217710, PubMed:25467981, PubMed:28504928). Interaction with PCDH15 is required for efficient localization to hair bundles (PubMed:23217710).SUBCELLULAR LOCATION Efficient localization to the plasma membrane requires the presence of PCDH15.TISSUE SPECIFICITY Brain, inner ear hair cells and vestibular neuroepithelia of the inner ear (PubMed:15905332, PubMed:16459341, PubMed:23217710). In inner ear, expressed in stereocilia in a punctate pattern and at the tip-link region (at protein level) (PubMed:15905332, PubMed:16459341, PubMed:23217710). Strongly expressed in brain (PubMed:26964900). Weakly expressed in heart, testis and intestine (PubMed:26964900).DEVELOPMENTAL STAGE Expressed in inner ear hair cells at 16.5 dpc. Expressed postnatally in inner and outer hair cells of the cochlear, as well as in vestibular hair cells. At the cochlear apex, levels are low at P1 and increased thereafter. After P7, hardly detectable at the protein level, while mRNA levels remains high in adult hair cells.DISEASE Defects in Lhfpl5 are the cause of the hurry-scurry (hscy) phenotype which is characterized by rapid circling behavior, frequent shaking of head from side to side, deafness and vestibular dysfunction.SIMILARITY Belongs to the LHFP family.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: fullName evidence="1"LHFPL tetraspan subfamily member 5 protein alternativeName: fullName evidence="12"Lipoma HMGIC fusion partner-like 5 protein alternativeName: fullName evidence="10"Tetraspan membrane protein of hair cell stereocilia
geneNameLhfpl5
Tmhs
identifierQ4KL25
isSequenceChangedFALSE
keyword3D-structure
Cell membrane
Disease variant
Ion transport
Membrane
Reference proteome
Transmembrane
Transmembrane helix
Transport
modified[InstanceEdit:354386] Schmidt, EE, 2008-06-18 04:45:12
[InstanceEdit:384350] Kanapin, AA, 2008-11-26 14:00:39
[InstanceEdit:392885] Kanapin, AA, 2009-03-09 12:07:18
[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
[InstanceEdit:423310] Kanapin, AA
[InstanceEdit:435478] Kanapin, AA
[InstanceEdit:435871] Kanapin, AA
[InstanceEdit:447347] Kanapin, AA
[InstanceEdit:525883] Kanapin, AA
[InstanceEdit:613449] Kanapin, AA
[InstanceEdit:797602] Kanapin, AA
[InstanceEdit:937368] Yung, CK
[InstanceEdit:1042053] Yung, CK
[InstanceEdit:1220657] Yung, CK
[InstanceEdit:1300696] Yung, CK
[InstanceEdit:1301627] Yung, CK
[InstanceEdit:1551960] Weiser, JD
[InstanceEdit:1995863] Weiser, JD
[InstanceEdit:2132304] Weiser, JD
[InstanceEdit:2265580] Weiser, JD
[InstanceEdit:4341137] Weiser, JD
[InstanceEdit:5433710] Weiser, JD
[InstanceEdit:5618415] Weiser, JD
[InstanceEdit:5634237] Weiser, JD
[InstanceEdit:5673015] Weiser, JD
[InstanceEdit:9027688] Weiser, JD
[InstanceEdit:9037114] Weiser, JD
[InstanceEdit:9627708] Weiser, JD
[InstanceEdit:9637257] Weiser, JD
[InstanceEdit:9676415] Weiser, JD
[InstanceEdit:9715482] Weiser, JD
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
nameLhfpl5
referenceDatabase[ReferenceDatabase:2] UniProt
secondaryIdentifierLHPL5_MOUSE
B2KF30
sequenceLength219
species[Species:48892] Mus musculus
(referenceEntity)[EntityWithAccessionedSequence:9658855] Lhfpl5 [plasma membrane] [Mus musculus]
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