File:Mouse organ of corti NeuroD1.jpg

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Neurod1 is necessary for development of an orderly patterned organ of Corti

(A, A’, C, C’, E) Myo VIIa immunocytochemistry shows upregulation of Myo VIIa in the wild-type starts around E16.5 from the mid-base and later progresses toward both base and apex with a regular organization of one row of inner and three rows of outer hair cells throughout the cochlea.

(B, B’, D) In contrast, Myo VIIa is already expressed throughout the cochlea with disorganization of hair cells in the apical half in E16.5 Neurod1 CKO mutant littermates.

In later stages, the basal half of the CKO mutant shows normal orientation of the hair cells (D, D’) whereas the apex of Neurod1 CKO mice shows multiple rows of both IHCs and OHCs with reduction of Myo VIIa intensity in most outer hair cells (F, F’). In addition, clusters of higher intensity of Myo VIIa positive cells are found in between outer hair cells with equivalent staining intensity to inner hair cells (arrows in F’).

The apical tip of the mutant cochlea shows a partially duplicated row of inner hair cells with complete absence of outer hair cells (F”). Using espin immunocytochemistry we confirm the disorganization of the apical half of the mutant cochlea where two rows of inner hair stereocilia and four to five rows of outer hair stereocilia are observed (H–H’) along with some unusually displaced strongly stained inner hair stereocilia (arrow in I) in between faintly labeled outer hair stereocilia (I). IHC, inner hair cells; OHC, outer hair cells.

Bar indicates 100 µm except F”; 10 µm in F”.


Links: inner ear | hearing | bHLH | mouse


Human bHLH Family  
Table - basic Helix Loop Helix Factor Family bHLH
Approved
Symbol 		Approved Name Previous
Symbols 		Synonyms Chromosome
NEUROD2 neuronal differentiation 2 bHLHa1, NDRF 17q12
NEUROD6 neuronal differentiation 6 Nex1, Math-2, bHLHa2, NEX1M, Atoh2 7p14.3
NEUROD1 neuronal differentiation 1 NEUROD MODY6, bHLHa3, NeuroD, BHF-1, BETA2 2q31.3
NEUROD4 neuronal differentiation 4 Atoh3, MATH-3, ATH-3, bHLHa4 12q13.13
NEUROG1 neurogenin 1 NEUROD3 bHLHa6, Math4C, ngn1, AKA 5q31.1
NEUROG3 neurogenin 3 Math4B, Atoh5, ngn3, bHLHa7 10q22.1
NEUROG2 neurogenin 2 Atoh4, bHLHa8, ngn-2, NGN2, Math4A 4q25
BHLHA9 basic helix-loop-helix family member a9 bHLHa9, BHLHF42 17p13.3
ATOH7 atonal bHLH transcription factor 7 Math5, bHLHa13 10q21.3
ATOH1 atonal bHLH transcription factor 1 MATH-1, bHLHa14, Math1, HATH1 4q22.2
BHLHA15 basic helix-loop-helix family member a15 BHLHB8 MIST1, bHLHa15 7q21.3
TAL1 TAL bHLH transcription factor 1, erythroid differentiation factor TCL5 SCL, bHLHa17 1p33
LYL1 LYL1, basic helix-loop-helix family member bHLHa18 19p13.13
TAL2 TAL bHLH transcription factor 2 bHLHa19 9q31.2
ATOH8 atonal bHLH transcription factor 8 bHLHa21, HATH6, FLJ14708 2p11.2
MSC musculin bHLHa22, ABF-1 8q13.3
TCF21 transcription factor 21 bHLHa23, POD1 6q23.2
TCF23 transcription factor 23 bHLHa24, OUT 2p23.3
TCF24 transcription factor 24 bHLHa25 8q13.1
HAND2 heart and neural crest derivatives expressed 2 bHLHa26, Thing2, Hed, dHand 4q34.1
HAND1 heart and neural crest derivatives expressed 1 Hxt, Thing1, eHand, bHLHa27 5q33.2
PTF1A pancreas associated transcription factor 1a p48, bHLHa29, PTF1-p48 10p12.2
PERD3L Fer3 like bHLH transcription factor N-TWIST, bHLHa31, NATO3 7p21.1
NHLH2 nescient helix-loop-helix 2 HEN2 bHLHa34, NSCL2 1p13.1
NHLH1 nescient helix-loop-helix 1 HEN1 bHLHa35, NSCL, NSCL1 1q23.2
TWIST1 twist family bHLH transcription factor 1 ACS3, BPES3, TWIST, CRS bHLHa38, H-twist, SCS, CRS1, BPES2 7p21.1
TWIST2 twist family bHLH transcription factor 2 bHLHa39, Dermo-1, DERMO1 2q37.3
TCF15 transcription factor 15 bHLHa40, PARAXIS, EC2 20p13
ASCL3 achaete-scute family bHLH transcription factor 3 HASH3, bHLHa42, Sgn1 11p15.4
ASCL4 achaete-scute family bHLH transcription factor 4 bHLHa44, HASH4 12q23.3
ASCL2 achaete-scute family bHLH transcription factor 2 ASH2, bHLHa45, HASH2 11p15.5
ASCL1 achaete-scute family bHLH transcription factor 1 bHLHa46, HASH1, ASH1 12q23.2
ASCL5 achaete-scute family bHLH transcription factor 5 bHLHa47 1q32.1
SCX scleraxis bHLH transcription factor SCXA, SCXB bHLHa41, bHLHa48 8q24.3
BHLHB9 basic helix-loop-helix family member b9 p60TRP, KIAA1701, GASP3 Xq22.1
USF1 upstream transcription factor 1 bHLHb11, UEF, MLTFI 1q23.3
USF2 upstream transcription factor 2, c-fos interacting FIP, bHLHb12 19q13
TCF4 transcription factor 4 SEF2-1B, ITF2, bHLHb19, E2-2 18q21.2
TCF12 transcription factor 12 HsT17266, p64, HTF4, bHLHb20, HEB 15q21.3
TCF3 transcription factor 3 MGC129647, bHLHb21, p75, E2A, VDIR, E47, MGC129648, ITF1 19p13.3
ID1 inhibitor of DNA binding 1, HLH protein bHLHb24, dJ857M17.1.2 20q11.21
ID3 inhibitor of DNA binding 3, HLH protein bHLHb25, HEIR-1 1p36.12
ID2 inhibitor of DNA binding 2 GIG8, bHLHb26 2p25.1
ID4 inhibitor of DNA binding 4, HLH protein bHLHb27 6p22.3
HEY1 hes related family bHLH transcription factor with YRPW motif 1 bHLHb31, CHF-2, HRT-1, HESR1, CHF2, HERP2, HESR-1 8q21.13
HEY2 hes related family bHLH transcription factor with YRPW motif 2 bHLHb32, HESR2, HERP1 6q22.31
HEYL hes related family bHLH transcription factor with YRPW motif-like HEY3, HESR3, bHLHb33 1p34.2
HES7 hes family bHLH transcription factor 7 bHLHb37 17p13.1
HES5 hes family bHLH transcription factor 5 bHLHb38 1p36.32
HES1 hes family bHLH transcription factor 1 HRY HES-1, Hes1, bHLHb39, FLJ20408 3q29
HES2 hes family bHLH transcription factor 2 bHLHb40 1p36.31
HES6 hes family bHLH transcription factor 6 bHLHb41 2q37.3
HES4 hes family bHLH transcription factor 4 bHLHb42 1p36.33
HES3 hes family bHLH transcription factor 3 bHLHb43 1p36.31
HELT helt bHLH transcription factor bHLHb44, HESL, HCM1228, MEGANE, Mgn 4q35.1
MYOD1 myogenic differentiation 1 MYF3 PUM, bHLHc1, MYOD 11p15.1
MYF5 myogenic factor 5 bHLHc2 12q21.31
MYOG myogenin MYF4 bHLHc3 1q32.1
MYF6 myogenic factor 6 MRF4, bHLHc4 12q21.31
MESP1 mesoderm posterior bHLH transcription factor 1 bHLHc5, MGC10676 15q26.1
MESP2 mesoderm posterior bHLH transcription factor 2 SCDO2, bHLHc6 15q26.1
FIGLA folliculogenesis specific bHLH transcription factor bHLHc8 2p13.3
MXI1 MAX interactor 1, dimerization protein MAD2, MXI, MXD2, bHLHc11 10q25.2
MXD4 MAX dimerization protein 4 bHLHc12, MST149, MSTP149, MAD4 4p16.3
MXD3 MAX dimerization protein 3 bHLHc13, MAD3 5q35.3
TFAP4 transcription factor AP-4 bHLHc41, AP-4 16p13.3
MXD1 MAX dimerization protein 1 MAD bHLHc58, MAD1 2p13.3
SREBF1 sterol regulatory element binding transcription factor 1 SREBP1a, SREBP1, SREBP-1c, bHLHd1 17p11.2
SREBF2 sterol regulatory element binding transcription factor 2 bHLHd2, SREBP2 22q13.2
MNT MAX network transcriptional repressor ROX, MXD6, MAD6, bHLHd3 17p13.3
MAX MYC associated factor X bHLHd7, bHLHd5, bHLHd8, bHLHd6, bHLHd4 14q23.3
MLX MLX, MAX dimerization protein TCFL4 bHLHd13, MXD7, MAD7 17q21.1
MLXIPL MLX interacting protein like WBSCR14 bHLHd14, MONDOB, MIO, WS-bHLH, CHREBP 7q11.23
ARNT2 aryl hydrocarbon receptor nuclear translocator 2 KIAA0307, bHLHe1 15q25.1
ARNT aryl hydrocarbon receptor nuclear translocator bHLHe2, HIF-1beta 1q21.3
ARNTL aryl hydrocarbon receptor nuclear translocator like PASD3, BMAL1, MOP3, JAP3, bHLHe5 11p15.3
ARNTL2 aryl hydrocarbon receptor nuclear translocator like 2 MOP9, bHLHe6, PASD9, CLIF, BMAL2 12p11.23
CLOCK clock circadian regulator KIAA0334, bHLHe8, KAT13D 4q12
NPAS2 neuronal PAS domain protein 2 PASD4, MOP4, bHLHe9 2q11.2
NPAS1 neuronal PAS domain protein 1 bHLHe11, MOP5, PASD5 19q13.32
NPAS3 neuronal PAS domain protein 3 MOP6, bHLHe12, PASD6 14q13.1
SIM1 SIM bHLH transcription factor 1 bHLHe14 6q16.3
SIM2 SIM bHLH transcription factor 2 SIM MGC119447, bHLHe15 21q22.13
HIF3A hypoxia inducible factor 3 subunit alpha MOP7, IPAS, bHLHe17, PASD7 19q13.32
OLIG2 oligodendrocyte transcription factor 2 PRKCBP2, BHLHB1 OLIGO2, RACK17, bHLHe19 21q22.11
OLIG3 oligodendrocyte transcription factor 3 bHLHe20, Bhlhb7 6q23.3
OLIG1 oligodendrocyte transcription factor 1 BHLHB6, bHLHe21 21q22.11
BHLHE22 basic helix-loop-helix family member e22 TNRC20, BHLHB5 CAGL85, Beta3, bHLHe22 8q12.3
BHLHE23 basic helix-loop-helix family member e23 BHLHB4 bA305P22.3, Beta4, bHLHe23 20q13.33
MITF melanogenesis associated transcription factor WS2A, WS2 bHLHe32, MI 3p13
TFE3 transcription factor binding to IGHM enhancer 3 TFEA, bHLHe33 Xp11.23
TFEC transcription factor EC bHLHe34, TCFEC, TFECL 7q31.2
TFEB transcription factor EB bHLHe35, TCFEB 6p21.1
MLXIP MLX interacting protein MIR, KIAA0867, bHLHe36, MONDOA 12q21.31
MYCN MYCN proto-oncogene, bHLH transcription factor NMYC bHLHe37, N-myc, MYCNOT 2p24.3
MYCL MYCL proto-oncogene, bHLH transcription factor MYCL1 bHLHe38, LMYC 1p34.2
MYC MYC proto-oncogene, bHLH transcription factor MYCC, bHLHe39, c-Myc 8q24.21
BHLHE40 basic helix-loop-helix family member e40 STRA13, BHLHB2 DEC1, bHLHe40, SHARP2, Clast5 3p26.1
BHLHE41 basic helix-loop-helix family member e41 BHLHB3 DEC2, SHARP-1, SHARP1, bHLHe41 12p12.1
NCOA3 nuclear receptor coactivator 3 SRC-3, KAT13B, CAGH16, ACTR, p/CIP, bHLHe42, TRAM-1, AIB1, TNRC16, SRC3, RAC3 20q13.12
EPAS1 endothelial PAS domain protein 1 HLF, HIF2A, bHLHe73, MOP2, PASD2 2p21
NCOA1 nuclear receptor coactivator 1 RIP160, bHLHe74, NCoA-1, F-SRC-1, SRC1, KAT13A 2p23.3
NCOA2 nuclear receptor coactivator 2 TIF2, bHLHe75, NCoA-2, GRIP1, KAT13C 8q13.3
AHR aryl hydrocarbon receptor bHLHe76 7p21.1
AHRR aryl-hydrocarbon receptor repressor AHH, AHHR KIAA1234, bHLHe77 5p15.33
HIF1A hypoxia inducible factor 1 subunit alpha bHLHe78, HIF1, HIF-1alpha, MOP1, PASD8 14q23.2
NPAS4 neuronal PAS domain protein 4 bHLHe79, Le-PAS, NXF, PASD10 11q13.2
SOHLH1 spermatogenesis and oogenesis specific basic helix-loop-helix 1 C9orf157 NOHLH, bA100C15.3, SPATA27, bHLHe80, TEB2 9q34.3
SOHLH2 spermatogenesis and oogenesis specific basic helix-loop-helix 2 TEB1, SPATA28, bHLHe81, FLJ20449 13q13.3
TCFL5 transcription factor like 5 CHA, E2BP-1, bHLHe82, Figlb 20q13.33
    Links: Developmental Signals - basic Helix-Loop-Helix | HGNC | Bmp Family | Fgf Family | Sox Family | Tbx Family

Reference

Jahan I, Pan N, Kersigo J & Fritzsch B. (2010). Neurod1 suppresses hair cell differentiation in ear ganglia and regulates hair cell subtype development in the cochlea. PLoS ONE , 5, e11661. PMID: 20661473 DOI.

Copyright

Copyright: © 2010 Jahan et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Figure 5. https://doi.org/10.1371/journal.pone.0011661.g005

Above text modified from original figure legend.


Cite this page: Hill, M.A. (2026, Mayıs 13) Embryology Mouse organ of corti NeuroD1.jpg. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/File:Mouse_organ_of_corti_NeuroD1.jpg

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current18:24, 30 July 2018Thumbnail for version as of 18:24, 30 July 20181,779 × 2,383 (1.11 MB)Z8600021 (talk | contribs)Figure 5. Neurod1 is necessary for development of an orderly patterned organ of Corti. Myo VIIa immunocytochemistry shows upregulation of Myo VIIa in the wild-type starts around E16.5 from the mid-base and later progresses toward both base and apex wit...

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