Gene Symbol: Col4a1
Description: collagen, type IV, alpha 1
Alias: Bru, Col4a-1, Raw, Svc, collagen alpha-1(IV) chain, alpha-1 type IV collagen, alpha1(IV) collagen, procollagen, type IV, alpha 1
Species: mouse
Products:     Col4a1

Top Publications

  1. Kelley P, Sado Y, Duncan M. Collagen IV in the developing lens capsule. Matrix Biol. 2002;21:415-23 pubmed
  2. Fox M, Sanes J, Borza D, Eswarakumar V, Fassler R, Hudson B, et al. Distinct target-derived signals organize formation, maturation, and maintenance of motor nerve terminals. Cell. 2007;129:179-93 pubmed
    ..Thus, multiple target-derived signals permit discrete control of the formation, maturation, and maintenance of presynaptic specializations. ..
  3. Graus Porta D, Blaess S, Senften M, Littlewood Evans A, Damsky C, Huang Z, et al. Beta1-class integrins regulate the development of laminae and folia in the cerebral and cerebellar cortex. Neuron. 2001;31:367-79 pubmed
    ..The phenotype of the beta1-deficient mice resembles pathological changes observed in human cortical dysplasias, suggesting that defective integrin-mediated signal transduction contributes to the development of some of these diseases. ..
  4. Thaung C, West K, Clark B, McKie L, Morgan J, Arnold K, et al. Novel ENU-induced eye mutations in the mouse: models for human eye disease. Hum Mol Genet. 2002;11:755-67 pubmed
    ..This collection of eye mutations will be valuable for understanding gene function, for dissecting protein function and as models of human eye disease. ..
  5. Van Agtmael T, Bailey M, Schlötzer Schrehardt U, Craigie E, Jackson I, Brownstein D, et al. Col4a1 mutation in mice causes defects in vascular function and low blood pressure associated with reduced red blood cell volume. Hum Mol Genet. 2010;19:1119-28 pubmed publisher
    Collagen type IV is the major structural component of the basement membrane and COL4A1 mutations cause adult small vessel disease, familial porencephaly and hereditary angiopathy with nephropathy aneurysm and cramps (HANAC) syndrome...
  6. Taylor S, Al Youha S, Van Agtmael T, Lu Y, Wong J, McGrouther D, et al. Tendon is covered by a basement membrane epithelium that is required for cell retention and the prevention of adhesion formation. PLoS ONE. 2011;6:e16337 pubmed publisher
    ..To evaluate the cell retentive properties of the BM in vivo we examined the tendons of the Col4a1(+/Svc) mouse that is heterozygous for a G-to-A transition in the Col4a1 gene that produces a G1064D substitution ..
  7. Jeanne M, Labelle Dumais C, Jorgensen J, Kauffman W, Mancini G, Favor J, et al. COL4A2 mutations impair COL4A1 and COL4A2 secretion and cause hemorrhagic stroke. Am J Hum Genet. 2012;90:91-101 pubmed publisher
    Collagen, type IV, alpha 1 (COL4A1) and alpha 2 (COL4A2) form heterotrimers and are abundant components of basement membranes, including those of the cerebral vasculature...
  8. Labelle Dumais C, Dilworth D, Harrington E, de Leau M, Lyons D, Kabaeva Z, et al. COL4A1 mutations cause ocular dysgenesis, neuronal localization defects, and myopathy in mice and Walker-Warburg syndrome in humans. PLoS Genet. 2011;7:e1002062 pubmed publisher
    ..we provide evidence that mutations in a gene coding for a major basement membrane protein, collagen IV alpha 1 (COL4A1), are a novel cause of MEB/WWS...
  9. Gould D, Phalan F, van Mil S, Sundberg J, Vahedi K, Massin P, et al. Role of COL4A1 in small-vessel disease and hemorrhagic stroke. N Engl J Med. 2006;354:1489-96 pubmed
    ..In this report, we show that a mutation in the mouse Col4a1 gene, encoding procollagen type IV alpha1, predisposes both newborn and adult mice to intracerebral hemorrhage...

More Information


  1. Van Agtmael T, Schlötzer Schrehardt U, McKie L, Brownstein D, Lee A, Cross S, et al. Dominant mutations of Col4a1 result in basement membrane defects which lead to anterior segment dysgenesis and glomerulopathy. Hum Mol Genet. 2005;14:3161-8 pubmed
    ..have identified an allelic series of three ENU induced dominant mouse mutants with missense mutations in the gene Col4a1 encoding the alpha1(IV) subunit chain...
  2. Kuo D, Labelle Dumais C, Mao M, Jeanne M, Kauffman W, Allen J, et al. Allelic heterogeneity contributes to variability in ocular dysgenesis, myopathy and brain malformations caused by Col4a1 and Col4a2 mutations. Hum Mol Genet. 2014;23:1709-22 pubmed publisher
    Collagen type IV alpha 1 and 2 (COL4A1 and COL4A2) are present in nearly all basement membranes...
  3. Firtina Z, Danysh B, Bai X, Gould D, Kobayashi T, Duncan M. Abnormal expression of collagen IV in lens activates unfolded protein response resulting in cataract. J Biol Chem. 2009;284:35872-84 pubmed publisher
    ..In Col4a1(+/Deltaex40) mutant mice, the accumulation of mutant chains also caused low levels of UPR activation...
  4. Gould D, Phalan F, Breedveld G, van Mil S, Smith R, Schimenti J, et al. Mutations in Col4a1 cause perinatal cerebral hemorrhage and porencephaly. Science. 2005;308:1167-71 pubmed
    ..We show that vascular defects are caused by a semidominant mutation in the procollagen type IV alpha 1 gene (Col4a1) in mice, which inhibits the secretion of mutant and normal type IV collagen...
  5. Bai X, Dilworth D, Weng Y, Gould D. Developmental distribution of collagen IV isoforms and relevance to ocular diseases. Matrix Biol. 2009;28:194-201 pubmed publisher
  6. Favor J, Gloeckner C, Janik D, Klempt M, Neuhäuser Klaus A, Pretsch W, et al. Type IV procollagen missense mutations associated with defects of the eye, vascular stability, the brain, kidney function and embryonic or postnatal viability in the mouse, Mus musculus: an extension of the Col4a1 allelic series and the identificatio. Genetics. 2007;175:725-36 pubmed
    ..Major components of the basement membrane include laminins and type IV collagens. The type IV procollagens Col4a1 and Col4a2 form the heterotrimer [alpha1(IV)]2[alpha2(IV)], which is ubiquitously expressed in basement membranes ..
  7. Gould D, Marchant J, Savinova O, Smith R, John S. Col4a1 mutation causes endoplasmic reticulum stress and genetically modifiable ocular dysgenesis. Hum Mol Genet. 2007;16:798-807 pubmed
    ..Here, we show that a mutation in the type IV collagen alpha 1 (Col4a1) gene can cause both ASD and optic nerve hypoplasia. COL4A1 is a major component of almost all basement membranes...
  8. Poschl E, Schlötzer Schrehardt U, Brachvogel B, Saito K, Ninomiya Y, Mayer U. Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development. Development. 2004;131:1619-28 pubmed
    ..for basement membrane assembly and embryonic development, we generated a null allele of the Col4a1/2 locus in mice, thereby ablating both alpha-chains. Unexpectedly, embryos developed up to E9...
  9. Aso S, Baba R, Noda S, Ikuno S, Fujita M. Hypoplastic basement membrane of the lens anlage in the inheritable lens aplastic mouse (lap mouse). Teratology. 2000;61:262-72 pubmed
    ..This result may indicate a possibility of the relationship between the basement membrane and apoptosis in this mutant. ..
  10. Miner J, Li C, Mudd J, Go G, Sutherland A. Compositional and structural requirements for laminin and basement membranes during mouse embryo implantation and gastrulation. Development. 2004;131:2247-56 pubmed
    ..These data suggest that both the molecular composition and the integrity of basement membranes are crucial for early developmental events. ..
  11. Favor J. The frequency of dominant cataract and recessive specific-locus mutations in mice derived from 80 or 160 mg ethylnitrosourea per kg body weight treated spermatogonia. Mutat Res. 1986;162:69-80 pubmed
    ..The imposition of the criterion that suspected variants be subjected to a genetic confirmation test has at least two advantages beside the fact that results represent unambiguous mutational events.(ABSTRACT TRUNCATED AT 400 WORDS) ..
  12. Loscertales M, Nicolaou F, Jeanne M, Longoni M, Gould D, Sun Y, et al. Type IV collagen drives alveolar epithelial-endothelial association and the morphogenetic movements of septation. BMC Biol. 2016;14:59 pubmed publisher
    ..We have performed a microarray expression analysis in late chick lung development and found that COL4A1 and COL4A2 were among the most significantly upregulated genes during the formation of the avian BGB...
  13. Sun J, Ting M, Ishii M, Maxson R. Msx1 and Msx2 function together in the regulation of primordial germ cell migration in the mouse. Dev Biol. 2016;417:11-24 pubmed publisher
    ..We also noted a reduction in the expression of Wnt5a and an increase in the expression in Bmp4 in such tissues in Msx1(-/-);Msx2(-/-) mutants, both known effectors of PGC development. ..
  14. Baek S, Tallquist M. Nf1 limits epicardial derivative expansion by regulating epithelial to mesenchymal transition and proliferation. Development. 2012;139:2040-9 pubmed publisher
    ..Our results demonstrate a regulatory role for Nf1 during epicardial EMT and provide insights into the susceptibility of patients with disrupted NF1 signaling to cardiovascular disease. ..
  15. Meyer zum Gottesberge A, Felix H. Abnormal basement membrane in the inner ear and the kidney of the Mpv17-/- mouse strain: ultrastructural and immunohistochemical investigations. Histochem Cell Biol. 2005;124:507-16 pubmed
    ..These studies explain the cellular responses to missing Mpv17-protein, such as accumulation of the extracellular matrix, degeneration, and apoptosis in the inner ear. ..
  16. Miner J, Li C. Defective glomerulogenesis in the absence of laminin alpha5 demonstrates a developmental role for the kidney glomerular basement membrane. Dev Biol. 2000;217:278-89 pubmed
  17. Wu J, Santoro S. Differential expression of integrin alpha subunits supports distinct roles during lung branching morphogenesis. Dev Dyn. 1996;206:169-81 pubmed
  18. Killen P, Burbelo P, Sakurai Y, Yamada Y. Structure of the amino-terminal portion of the murine alpha 1(IV) collagen chain and the corresponding region of the gene. J Biol Chem. 1988;263:8706-9 pubmed
    ..The exon structure for the helical portion of the 7 S domain is different from that of the major helical domain, suggesting that they evolved differently. ..
  19. Burbelo P, Martin G, Yamada Y. Alpha 1(IV) and alpha 2(IV) collagen genes are regulated by a bidirectional promoter and a shared enhancer. Proc Natl Acad Sci U S A. 1988;85:9679-82 pubmed
  20. Bhatt R, Tomoda T, Fang Y, Hatten M. Discoidin domain receptor 1 functions in axon extension of cerebellar granule neurons. Genes Dev. 2000;14:2216-28 pubmed
    ..Our results therefore indicate that collagen-DDR1 signaling is essential for granule neuron axon formation and further suggest a unique role of pia in cerebellar cortex histogenesis. ..
  21. Favor J, Neuhauser Klaus A. Saturation mutagenesis for dominant eye morphological defects in the mouse Mus musculus. Mamm Genome. 2000;11:520-5 pubmed
  22. Clifford J, Menter D, Wang M, Lotan R, Lippman S. Retinoid receptor-dependent and -independent effects of N-(4-hydroxyphenyl)retinamide in F9 embryonal carcinoma cells. Cancer Res. 1999;59:14-8 pubmed
    ..Therefore, two distinct effects of 4HPR were identified in this system: a rapid induction of cell death and a slower induction of differentiation, which are likely to be receptor independent and dependent, respectively...
  23. Kurahashi H, Taniguchi M, Meno C, Taniguchi Y, Takeda S, Horie M, et al. Basement membrane fragility underlies embryonic lethality in fukutin-null mice. Neurobiol Dis. 2005;19:208-17 pubmed
    ..BM fragility may underlie all of these abnormal phenotypes, and maintenance of BM function may require fukutin-mediated glycosylation of alpha-dystroglycan early in embryonic development. ..
  24. Ma S, Santhosh D, Kumar T P, Huang Z. A Brain-Region-Specific Neural Pathway Regulating Germinal Matrix Angiogenesis. Dev Cell. 2017;41:366-381.e4 pubmed publisher
    ..They also identify tissue-specific molecular targets for GM hemorrhage intervention. ..
  25. Filie J, Burbelo P, Kozak C. Genetic mapping of the alpha 1 and alpha 2 (IV) collagen genes to mouse chromosome 8. Mamm Genome. 1995;6:487 pubmed
  26. French W, Creemers E, Tallquist M. Platelet-derived growth factor receptors direct vascular development independent of vascular smooth muscle cell function. Mol Cell Biol. 2008;28:5646-57 pubmed publisher
    ..Together, these data demonstrate that PDGF receptors cooperate in the yolk sac mesothelium to direct blood vessel maturation and suggest that these effects are independent of their role in VSMC development...
  27. Pietilä I, Prunskaite Hyyryläinen R, Kaisto S, Tika E, van Eerde A, Salo A, et al. Wnt5a Deficiency Leads to Anomalies in Ureteric Tree Development, Tubular Epithelial Cell Organization and Basement Membrane Integrity Pointing to a Role in Kidney Collecting Duct Patterning. PLoS ONE. 2016;11:e0147171 pubmed publisher
    ..Together Wnt5a has a novel function in kidney organogenesis by contributing to patterning of UB derived collecting duct development contributing putatively to congenital disease. ..
  28. Park J, Kato M, Lanting L, Castro N, Nam B, Wang M, et al. Repression of let-7 by transforming growth factor-β1-induced Lin28 upregulates collagen expression in glomerular mesangial cells under diabetic conditions. Am J Physiol Renal Physiol. 2014;307:F1390-403 pubmed publisher
    ..of mesangial extracellular matrix (ECM) proteins such as collagen type 1-α2 (Col1a2) and collagen type 4-α1 (Col4a1) is a key feature of diabetic nephropathy (DN)...
  29. Sudhakar Y, Verma R, Pawar S. Type IV collagen ?1-chain noncollagenous domain blocks MMP-2 activation both in-vitro and in-vivo. Sci Rep. 2014;4:4136 pubmed publisher
    ..In-vivo studies using ?1-integrin null-mice treated with higher doses of ?1(IV)NC1 showed integrin independent inhibition of tumor growth and active-MMP-2, without affecting MMP-9, MMP-7 and angiostatin. ..
  30. Hanson K, Jung J, Tran Q, Hsu S, Iida R, Ajeti V, et al. Spatial and temporal analysis of extracellular matrix proteins in the developing murine heart: a blueprint for regeneration. Tissue Eng Part A. 2013;19:1132-43 pubmed publisher
    ..Similarly, fabricated scaffolds generated using ECM components, could be utilized for ventricular repair. ..
  31. Mokkapati S, Baranowsky A, Mirancea N, Smyth N, Breitkreutz D, Nischt R. Basement membranes in skin are differently affected by lack of nidogen 1 and 2. J Invest Dermatol. 2008;128:2259-67 pubmed publisher
    ..In summary, our results indicate that in skin the laminin composition of the various BMs determines whether nidogens are required for their assembly and stabilization. ..
  32. Manson S, Niederhoff R, Hruska K, Austin P. Endogenous BMP-7 is a critical molecular determinant of the reversibility of obstruction-induced renal injuries. Am J Physiol Renal Physiol. 2011;301:F1293-302 pubmed publisher
    ..Accordingly, renal recovery from obstructive uropathies may be optimized through timely intervention and adjuvant approaches to restore BMP-7 activity. ..
  33. Sun X, Pons S, Wang L, Zhang Y, Yenush L, Burks D, et al. The IRS-2 gene on murine chromosome 8 encodes a unique signaling adapter for insulin and cytokine action. Mol Endocrinol. 1997;11:251-62 pubmed
    ..Thus, signaling specificity through the IRS proteins may be accomplished by specific expression patterns and distinct phosphorylation patterns during interaction with various activated receptors. ..
  34. Yuan B, Li C, Kimura S, Engelhardt R, Smith B, Minoo P. Inhibition of distal lung morphogenesis in Nkx2.1(-/-) embryos. Dev Dyn. 2000;217:180-90 pubmed
    ..1 independent, while distal lung morphogenesis appears to be strictly dependent on the wild-type activity of Nkx2.1. ..
  35. Nakajima Y, Morishima M, Nakazawa M, Momma K, Nakamura H. Distribution of fibronectin, type I collagen, type IV collagen, and laminin in the cardiac jelly of the mouse embryonic heart with retinoic acid-induced complete transposition of the great arteries. Anat Rec. 1997;249:478-85 pubmed
    ..This may be one of the reasons why mesenchymal cell formation is inhibited in the RA-induced TGA. ..
  36. Omoteyama K, Mikami Y, Takagi M. Foxc2 induces expression of MyoD and differentiation of the mouse myoblast cell line C2C12. Biochem Biophys Res Commun. 2007;358:885-9 pubmed
    ..components in endothelial cells, we found no evidence of Foxc2-mediated regulation of Collagen type IV alpha 1 (Col4a1) or Col4a2 in myoblast cells...
  37. Steenhard B, Isom K, Stroganova L, St John P, Zelenchuk A, Freeburg P, et al. Deletion of von Hippel-Lindau in glomerular podocytes results in glomerular basement membrane thickening, ectopic subepithelial deposition of collagen {alpha}1{alpha}2{alpha}1(IV), expression of neuroglobin, and proteinuria. Am J Pathol. 2010;177:84-96 pubmed publisher
    ..We conclude that podocyte VHL is required for normal maintenance of podocytes, GBM composition and ultrastructure, and glomerular barrier properties. ..
  38. Rudat C, Norden J, Taketo M, Kispert A. Epicardial function of canonical Wnt-, Hedgehog-, Fgfr1/2-, and Pdgfra-signalling. Cardiovasc Res. 2013;100:411-21 pubmed publisher
    ..Canonical Wnt-, Hh-, and Fgfr1/Fgfr2-signalling are dispensable for epicardial development, but Pdgfra-signalling is crucial for the differentiation of cardiac fibroblasts from epicardium-derived cells. ..
  39. Chen S, Lin J, Lin H, Shan Y, Cheng Y, Yang B. Dependence of fibroblast infiltration in tumor stroma on type IV collagen-initiated integrin signal through induction of platelet-derived growth factor. Biochim Biophys Acta. 2015;1853:929-39 pubmed publisher
    ..The integrin signal pathway was activated by collagen engagement through Src and ERK, leading to enhanced PDGF-A production, which serves as a key regulator of fibroblast recruitment. ..
  40. Oberbäumer I. New pUC-derived expression vectors for rapid construction of cDNA libraries. Gene. 1986;49:81-91 pubmed
    ..Cell. Biol. 2 (1982) 161-170] method. We have successfully used this system to obtain cDNA clones coding for the different chains of the large basement membrane proteins type IV collagen and laminin. ..
  41. Munro D, Hohenstein P, Coate T, Davies J. Refuting the hypothesis that semaphorin-3f/neuropilin-2 exclude blood vessels from the cap mesenchyme in the developing kidney. Dev Dyn. 2017;246:1047-1056 pubmed publisher
    ..These results should provoke additional experiments to ascertain the biological significance of Sema3f/Nrp2 expression in the developing kidney. Developmental Dynamics 246:1047-1056, 2017. © 2017 Wiley Periodicals, Inc. ..
  42. Saika S, Liu C, Azhar M, Sanford L, Doetschman T, Gendron R, et al. TGFbeta2 in corneal morphogenesis during mouse embryonic development. Dev Biol. 2001;240:419-32 pubmed
    ..Delayed appearance of macrophages in ocular tissues was observed in Tgfb2(-/-) mice. Malfunctioning macrophages may account for accumulation of cell mass in vitreous of Tgfb2 null mice. ..
  43. Kurkinen M, Barlow D, Helfman D, Williams J, Hogan B. Isolation of cDNA clones for basal lamina components: type IV procollagen. Nucleic Acids Res. 1983;11:6199-209 pubmed
    ..Here we show, using one of the cDNA clones as a probe for type IV procollagen, that an increase in cellular concentration of type IV procollagen mRNA occurs within 24 to 48 hours of induction, reaching a constant high level by 72 hours. ..
  44. Braghetta P, Fabbro C, Piccolo S, Marvulli D, Bonaldo P, Volpin D, et al. Distinct regions control transcriptional activation of the alpha1(VI) collagen promoter in different tissues of transgenic mice. J Cell Biol. 1996;135:1163-77 pubmed
  45. Marin M, Karis A, Visser P, Grosveld F, Philipsen S. Transcription factor Sp1 is essential for early embryonic development but dispensable for cell growth and differentiation. Cell. 1997;89:619-28 pubmed
    ..MeCP2 is thought to be required for the maintenance of differentiated cells. We suggest that Sp1 is an important regulator of this process. ..
  46. Trouillet A, Lorach H, Dubus E, El Mathari B, Ivkovic I, Dégardin J, et al. Col4a1 mutation generates vascular abnormalities correlated with neuronal damage in a mouse model of HANAC syndrome. Neurobiol Dis. 2017;100:52-61 pubmed publisher
    ..It recapitulates most of the aspects of the human disease and is therefore a great tool to study and to address this condition. ..
  47. Kurkinen M, Condon M, Blumberg B, Barlow D, Quinones S, Saus J, et al. Extensive homology between the carboxyl-terminal peptides of mouse alpha 1(IV) and alpha 2(IV) collagen. J Biol Chem. 1987;262:8496-9 pubmed
    ..Presumably, they were first derived by internal duplication of a common ancestral DNA sequence which later, by gene duplication, gave rise to the two different but homologous carboxyl-terminal peptides of type IV collagen. ..
  48. Hiramatsu R, Matsuoka T, Kimura Yoshida C, Han S, Mochida K, Adachi T, et al. External mechanical cues trigger the establishment of the anterior-posterior axis in early mouse embryos. Dev Cell. 2013;27:131-144 pubmed publisher
  49. Takeoka H, Iehara N, Uematsu Yanagita M, Abe H, Sunamoto M, Yamada Y, et al. A multifunctional transcription factor (A1p145) regulates the smooth muscle phenotype in mesangial cells. Biochem Biophys Res Commun. 1998;252:290-5 pubmed
    ..The levels of proliferating cell nuclear antigen mRNA were also suppressed. These results suggest that A1p145 is a negative transcription factor for COL4 and may be a phenotypic modulator. ..
  50. Wu J, Santoro S. Complex patterns of expression suggest extensive roles for the alpha 2 beta 1 integrin in murine development. Dev Dyn. 1994;199:292-314 pubmed
    ..The developmental expression pattern of the alpha 2 beta 1 integrin suggests roles for the integrin in placental development and matrix assembly and remodeling. ..
  51. Boylan J, Lohnes D, Taneja R, Chambon P, Gudas L. Loss of retinoic acid receptor gamma function in F9 cells by gene disruption results in aberrant Hoxa-1 expression and differentiation upon retinoic acid treatment. Proc Natl Acad Sci U S A. 1993;90:9601-5 pubmed
    ..Our results support the idea that each RAR may regulate different subsets of RA-responsive genes, which may explain, in part, the complex regulation of developmental processes by retinoids. ..
  52. Williamson R, Henry M, Daniels K, Hrstka R, Lee J, Sunada Y, et al. Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice. Hum Mol Genet. 1997;6:831-41 pubmed
    ..Furthermore, these results suggest that disruption of basement membrane organization might be a common feature of muscular dystrophies linked to the DGC. ..
  53. Kiyozumi D, Osada A, Sugimoto N, Weber C, Ono Y, Imai T, et al. Identification of a novel cell-adhesive protein spatiotemporally expressed in the basement membrane of mouse developing hair follicle. Exp Cell Res. 2005;306:9-23 pubmed
    ..These results, together, indicate that QBRICK is an adhesive ligand of basement membrane distinctively recognized by cells in the embryonic skin and hair follicles through different types of integrins directed to the Arg-Gly-Asp motif. ..
  54. Oberbäumer I, Laurent M, Schwarz U, Sakurai Y, Yamada Y, Vogeli G, et al. Amino acid sequence of the non-collagenous globular domain (NC1) of the alpha 1(IV) chain of basement membrane collagen as derived from complementary DNA. Eur J Biochem. 1985;147:217-24 pubmed
    ..These DNA and protein sequences are compared to the corresponding sequences of other collagens and discussed with respect to their structural and biological significance. ..
  55. Borza C, Su Y, Tran T, Yu L, Steyns N, Temple K, et al. Discoidin domain receptor 1 kinase activity is required for regulating collagen IV synthesis. Matrix Biol. 2017;57-58:258-271 pubmed publisher
    ..In conclusion, our studies indicate that the kinase activity of DDR1 plays a key role in DDR1-induced collagen synthesis and suggest that blocking collagen-mediated DDR1 activation may be beneficial in fibrotic diseases. ..
  56. Kuncio G, Alvarez R, Li S, Killen P, Neilson E. Transforming growth factor-beta modulation of the alpha 1(IV) collagen gene in murine proximal tubular cells. Am J Physiol. 1996;271:F120-5 pubmed
    ..The activity of the alpha 1(IV) collagen gene in proximal tubular cells is increased by TGF-beta, which acts on the domain(s) embedded within the intergenic bidirectional promoter...
  57. Gilmour D, Lyon G, Carlton M, Sanes J, Cunningham J, Anderson J, et al. Mice deficient for the secreted glycoprotein SPARC/osteonectin/BM40 develop normally but show severe age-onset cataract formation and disruption of the lens. EMBO J. 1998;17:1860-70 pubmed
    ..The lens capsule, however, shows no qualitative changes in the major basal lamina proteins laminin, collagen IV, perlecan or entactin. These mice are an excellent resource for further studies on how SPARC affects cell behaviour in vivo. ..
  58. Smits P, Lefebvre V. Sox5 and Sox6 are required for notochord extracellular matrix sheath formation, notochord cell survival and development of the nucleus pulposus of intervertebral discs. Development. 2003;130:1135-48 pubmed
    ..Through these roles and essential roles in cartilage formation, they are central transcriptional regulators of vertebral column development. ..
  59. Arai K, Nishiyama T. Developmental changes in extracellular matrix messenger RNAs in the mouse placenta during the second half of pregnancy: possible factors involved in the regulation of placental extracellular matrix expression. Biol Reprod. 2007;77:923-33 pubmed
    Expression of procollagens (Col1a1/2, Col3a1, Col4a1/2, Col5a1/2) and fibronectin 1 (Fn1) in the mouse fetal placental tissue was examined during the second half of pregnancy...
  60. Barnette D, VandeKopple M, Wu Y, Willoughby D, Lincoln J. RNA-seq analysis to identify novel roles of scleraxis during embryonic mouse heart valve remodeling. PLoS ONE. 2014;9:e101425 pubmed publisher
    ..In summary, our findings have identified transcriptome profiles from abnormal heart valves isolated from E15.5 Scx-/- embryos that could be used in the future to understand mechanisms of heart valve disease in the human population. ..
  61. Lozanoff S, Johnston J, Ma W, Jourdan Le Saux C. Immunohistochemical localization of Pax2 and associated proteins in the developing kidney of mice with renal hypoplasia. J Histochem Cytochem. 2001;49:1081-97 pubmed
    ..J Histochem Cytochem 49:1081-1097, 2001) ..
  62. Valenzuela D, Griffiths J, Rojas J, Aldrich T, Jones P, Zhou H, et al. Angiopoietins 3 and 4: diverging gene counterparts in mice and humans. Proc Natl Acad Sci U S A. 1999;96:1904-9 pubmed
    ..Angiopoietin-3 and angiopoietin-4 have very different distributions in their respective species, and angiopoietin-3 appears to act as an antagonist, whereas angiopoietin-4 appears to function as an agonist. ..
  63. Karl J, Capel B. Sertoli cells of the mouse testis originate from the coelomic epithelium. Dev Biol. 1998;203:323-33 pubmed
    ..Taken together, our data show that the coelomic epithelium is a source of Sertoli cells as well as other somatic cells of the gonad in the developing mouse testis. ..
  64. Koizumi T, Hendel E, Lalley P, Tchetgen M, Nadeau J. Homologs of genes and anonymous loci on human chromosome 13 map to mouse chromosomes 8 and 14. Mamm Genome. 1995;6:263-8 pubmed
    ..Of the clones for four genes and twelve anonymous loci tested, cross-hybridization was found for six, COL4A1, COL4A2, D13S26, D13S35, F10, and PCCA...
  65. Sarthy V. Collagen IV mRNA expression during development of the mouse retina: an in situ hybridization study. Invest Ophthalmol Vis Sci. 1993;34:145-52 pubmed
    ..Retinal collagen IV is possibly derived from nonretinal sources, such as the lens, or more likely from the hyaloid vessels. ..
  66. Thomas T, Dziadek M. Genes coding for basement membrane glycoproteins laminin, nidogen, and collagen IV are differentially expressed in the nervous system and by epithelial, endothelial, and mesenchymal cells of the mouse embryo. Exp Cell Res. 1993;208:54-67 pubmed
  67. Sakurai Y, Sullivan M, Yamada Y. Alpha 1 type IV collagen gene evolved differently from fibrillar collagen genes. J Biol Chem. 1986;261:6654-7 pubmed
    ..These sizes do not appear to be related to the 54-base pair coding unit which is characteristic of fibrillar collagen exons, suggesting that the alpha 1 type IV collagen gene evolved differently from the fibrillar collagen genes.
  68. Shindo T, Manabe I, Fukushima Y, Tobe K, Aizawa K, Miyamoto S, et al. Krüppel-like zinc-finger transcription factor KLF5/BTEB2 is a target for angiotensin II signaling and an essential regulator of cardiovascular remodeling. Nat Med. 2002;8:856-63 pubmed
    ..KLF5 thus seems to be a key element linking external stress and cardiovascular remodeling. ..
  69. Yonezawa T, Hattori S, Inagaki J, Kurosaki M, Takigawa T, Hirohata S, et al. Type IV collagen induces expression of thrombospondin-1 that is mediated by integrin alpha1beta1 in astrocytes. Glia. 2010;58:755-67 pubmed publisher
    ..Collectively, our results suggest that vascular basement membrane components substantially impact gene expression in astrocytes during brain tissue repair. ..
  70. Huttner K, Kozak C, Bevins C. The mouse genome encodes a single homolog of the antimicrobial peptide human beta-defensin 1. FEBS Lett. 1997;413:45-9 pubmed
    ..These data indicate that mBD-1 is a close homolog of hBD-1, and suggest that analysis of its role in mouse host defense may provide significant insights into human epithelial innate immunity. ..
  71. Kaytes P, Wood L, Theriault N, Kurkinen M, Vogeli G. Head-to-head arrangement of murine type IV collagen genes. J Biol Chem. 1988;263:19274-7 pubmed
    ..These findings agree with linkage studies of these two genes and differ from the known organization of human fibrillar collagen genes which have been found to be dispersed within the human genome. ..
  72. Kratochvilova J, Favor J, Neuhauser Klaus A. Dominant cataract and recessive specific-locus mutations detected in offspring of procarbazine-treated male mice. Mutat Res. 1988;198:295-301 pubmed
    ..The viability and fertility of the heterozygotes of all 3 mutations were not affected. Only 1 mutation was shown to be viable as a homozygote. ..
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