Gene Symbol: Hook1
Description: hook microtubule tethering protein 1
Alias: A930033L17Rik, azh, protein Hook homolog 1, abnormal spermatozoon head shape, hook homolog 1, mHK1
Species: mouse
Products:     Hook1

Top Publications

  1. Meistrich M, Trostle Weige P, Womack J. Mapping of the azh locus to mouse chromosome 4. J Hered. 1992;83:56-61 pubmed
    The azh (abnormal spermatozoon headshape) mutation in the mouse, which results in abnormal sperm head formation, was demonstrated to display an autosomal recessive pattern of inheritance...
  2. Meistrich M, Kasai K, Olds Clarke P, MacGregor G, Berkowitz A, Tung K. Deficiency in fertilization by morphologically abnormal sperm produced by azh mutant mice. Mol Reprod Dev. 1994;37:69-77 pubmed
    Male mice homozygous for the azh mutation produce spermatozoa with abnormal head shapes and have significantly reduced fecundity, to between 5% and 10% that of wild-type or heterozygous mice...
  3. Schumacher J, Artzt K, Braun R. Spermatid perinuclear ribonucleic acid-binding protein binds microtubules in vitro and associates with abnormal manchettes in vivo in mice. Biol Reprod. 1998;59:69-76 pubmed
    ..We show here that despite the highly abnormal manchettes and microtubule aggregates formed in azh, hop-sterile, tw2, and tw8 mutants, SPNR remains associated with the manchettes...
  4. Akutsu H, Tres L, Tateno H, Yanagimachi R, Kierszenbaum A. Offspring from normal mouse oocytes injected with sperm heads from the azh/azh mouse display more severe sperm tail abnormalities than the original mutant. Biol Reprod. 2001;64:249-56 pubmed
    Sperm with abnormalities in the position and shape of the head were obtained from the azh/azh mutant and injected into the cytoplasm of mature mouse oocytes to determine whether sperm from the offspring display both head (club shape) and ..
  5. Zhou J, Du Y, Qin W, Hu Y, Huang Y, Bao L, et al. RIM-BP3 is a manchette-associated protein essential for spermiogenesis. Development. 2009;136:373-82 pubmed publisher
    ..Consistent with its role in morphogenesis, the RIM-BP3 protein physically associates with Hook1, a known manchette-bound protein required for sperm head morphogenesis...
  6. Schwarz T, Prieler B, Schmid J, Grzmil P, Neesen J. Ccdc181 is a microtubule-binding protein that interacts with Hook1 in haploid male germ cells and localizes to the sperm tail and motile cilia. Eur J Cell Biol. 2017;96:276-288 pubmed publisher
    Disruption of murine Hook1 results in a disturbed spermatogenesis and consequently leads to male infertility in mice...
  7. Russell L, Russell J, MacGregor G, Meistrich M. Linkage of manchette microtubules to the nuclear envelope and observations of the role of the manchette in nuclear shaping during spermiogenesis in rodents. Am J Anat. 1991;192:97-120 pubmed
    ..In addition, sys and azh mutant mice were used to determine the consequences of defective manchette formation...
  8. Meistrich M, Trostle Weige P, Russell L. Abnormal manchette development in spermatids of azh/azh mutant mice. Am J Anat. 1990;188:74-86 pubmed
    A study of manchette development during spermiogenesis in azh/azh mutant mice was carried out by thin-section transmission electron microscopy with the goal of determining which of the initial steps in spermatid development are aberrant...
  9. Mochida K, Tres L, Kierszenbaum A. Structural and biochemical features of fractionated spermatid manchettes and sperm axonemes of the azh/azh mutant mouse. Mol Reprod Dev. 1999;52:434-44 pubmed
    ..The nature of their molecular components and developmental sequence are not completely known. The azh/azh (for abnormal sperm headshape) mouse mutant is an ideal model for analyzing tubulin isotypes and microtubule-..

More Information


  1. Mendoza Lujambio I, Burfeind P, Dixkens C, Meinhardt A, Hoyer Fender S, Engel W, et al. The Hook1 gene is non-functional in the abnormal spermatozoon head shape (azh) mutant mouse. Hum Mol Genet. 2002;11:1647-58 pubmed
    In mice carrying the autosomal recessive mutation 'abnormal spermatozoon head shape' (azh) all spermatozoa display a highly abnormal head morphology that differs drastically from the compact and hook-shaped head of the normal murine sperm...
  2. Nayernia K, Vauti F, Meinhardt A, Cadenas C, Schweyer S, Meyer B, et al. Inactivation of a testis-specific Lis1 transcript in mice prevents spermatid differentiation and causes male infertility. J Biol Chem. 2003;278:48377-85 pubmed
    ..These results provide evidence for an essential and hitherto uncharacterized role of the Lis1 protein in spermatogenesis, particularly in the differentiation of spermatids into spermatozoa. ..
  3. Yamauchi Y, Ward M. Preservation of ejaculated mouse spermatozoa from fertile C57BL/6 and infertile Hook1/Hook1 mice collected from the uteri of mated females. Biol Reprod. 2007;76:1002-8 pubmed
    ..Here we obtained multiple samples of ejaculated spermatozoa from normal fertile C57BL/6 and infertile Hook1/Hook1 (formerly known as azh/azh) mutant males from uteri after mating, thus avoiding termination of the males...
  4. Appler J, Lu C, Druckenbrod N, Yu W, Koundakjian E, Goodrich L. Gata3 is a critical regulator of cochlear wiring. J Neurosci. 2013;33:3679-91 pubmed publisher
    ..As the first auditory-specific regulator of SGN development, Gata3 provides a useful molecular entry point for efforts to engineer SGNs for the restoration of hearing. ..
  5. Weimer J, Chattopadhyay S, Custer A, Pearce D. Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1. Biochem Biophys Res Commun. 2005;330:1176-81 pubmed
    b>Hook1 is a member of a family of microtubule-binding proteins. Studies on the Drosophila homolog of Hook1 have suggested a role in the maturation and trafficking of internalized proteins to the late endosome...
  6. Moreno R, Palomino J, Schatten G. Assembly of spermatid acrosome depends on microtubule organization during mammalian spermiogenesis. Dev Biol. 2006;293:218-27 pubmed
    ..The microtubule network of round spermatids of azh/azh mice also became disorganized...
  7. Olenick M, Tokito M, Boczkowska M, Dominguez R, Holzbaur E. Hook Adaptors Induce Unidirectional Processive Motility by Enhancing the Dynein-Dynactin Interaction. J Biol Chem. 2016;291:18239-51 pubmed publisher
    ..Here we investigate the role of mammalian Hook proteins, Hook1 and Hook3, as potential motor adaptors...