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Gene Review

GAR1  -  GAR1 ribonucleoprotein

Homo sapiens

Synonyms: H/ACA ribonucleoprotein complex subunit 1, NOLA1, Nucleolar protein family A member 1, snoRNP protein GAR1
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Disease relevance of NOLA1

  • Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1 [1].

High impact information on NOLA1

  • Here, we describe the 2.1 A crystal structure of a specific complex of three archaeal H/ACA proteins, Cbf5, Nop10, and Gar1 [2].
  • In eukaryotes, two distinct classes of small nucleolar RNAs (snoRNAs), namely the fibrillarin-associated box C/D snoRNAs and the Gar1p-associated box H/ACA snoRNAs, direct the site-specific 2'-O-ribose methylation and pseudouridylation of ribosomal RNAs (rRNAs), respectively [3].
  • Here we present results that indicate that the interaction of SMN with GAR1 is mediated by the Tudor domain of SMN [1].
  • Furthermore, we find that either of the two arginine/glycine-rich domains of GAR1 can provide for interaction with SMN, but removal of both results in loss of the interaction [1].
  • Single point mutations within the Tudor domain, including a spinal muscular atrophy patient mutation, impair the interaction of SMN with GAR1 [1].

Biological context of NOLA1

  • Using PCR, RNA blot and genomic DNA analysis, we provide evidence that the beta subunit is produced from a complex locus on chromosome 16 which is also capable of generating independent transcripts corresponding to GAR1 and the C-terminal two-thirds of the beta subunit [4].
  • Pretreatment with NOLA partially restored Ang II responses to postpartum levels [5].
  • After NOLA, acetylcholine-induced vasodilatation in the collared vascular bed was no longer different from the vasodilatation in the control bed.(ABSTRACT TRUNCATED AT 250 WORDS)[6]
  • 3. Oral NL-nitro-L-arginine (NOLA; 5 or 10 mg/kg) was given for 1 week in early (6-8 weeks gestation), middle (14-16 weeks gestation) and late (22-24 weeks gestation) pregnancy and while non-pregnant [7].

Anatomical context of NOLA1


Associations of NOLA1 with chemical compounds

  • GAR2, an aldehyde reductase, was also active in the reduction of D-glucose, with an apparent Km comparable to that of GAR1 but with a Vmax only 14% that of GAR1 [10].
  • Sodium depletion also significantly reduced responses to Ang II by the same amount as in pregnancy, and these responses returned to normal with pretreatment with NOLA [5].
  • Vascular NO production was inhibited by pretreatment with N omega-nitro-L-arginine (NOLA 10 mg/kg), a NO-synthase inhibitor [5].
  • The vasodilator responses to intravenous infusions of the endothelium-dependent vasodilator, acetylcholine, and glyceryl trinitrate were measured in each artery at 2 and 7 days after surgical placement of the collar, and again following infusion of the nitric oxide synthase inhibitor, N-nitro-L-arginine (NOLA, 15 mg/kg) [6].
  • Pretreatment with the cyclooxygenase inhibitor, indomethacin (1 microM) and the nitric oxide synthase inhibitor N(w)-nitro-L-arginine (NOLA, 100 microM) did not significantly affect either the EC(50) or the maximum contraction to ET-1 in veins from normal full-term and preterm preparations [11].

Other interactions of NOLA1

  • Coimmunoprecipitations demonstrate that the SMN complex associates with fibrillarin and with GAR1 in vivo [8].


  1. Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1. Whitehead, S.E., Jones, K.W., Zhang, X., Cheng, X., Terns, R.M., Terns, M.P. J. Biol. Chem. (2002) [Pubmed]
  2. Crystal structure of a Cbf5-Nop10-Gar1 complex and implications in RNA-guided pseudouridylation and dyskeratosis congenita. Rashid, R., Liang, B., Baker, D.L., Youssef, O.A., He, Y., Phipps, K., Terns, R.M., Terns, M.P., Li, H. Mol. Cell (2006) [Pubmed]
  3. A small nucleolar guide RNA functions both in 2'-O-ribose methylation and pseudouridylation of the U5 spliceosomal RNA. Jády, B.E., Kiss, T. EMBO J. (2001) [Pubmed]
  4. The beta subunit of human rod photoreceptor cGMP-gated cation channel is generated from a complex transcription unit. Ardell, M.D., Aragon, I., Oliveira, L., Porche, G.E., Burke, E., Pittler, S.J. FEBS Lett. (1996) [Pubmed]
  5. Role of nitric oxide in the attenuated pressor responses of pregnant or sodium-deplete sheep. Tresham, J.J., Boutros, P., Wintour, E.M., May, C.N. J. Cardiovasc. Pharmacol. (1996) [Pubmed]
  6. Impaired vasodilator function of nitric oxide associated with developing neo-intima in conscious rabbits. Arthur, J.F., Dusting, G.J., Woodman, O.L. J. Vasc. Res. (1994) [Pubmed]
  7. Low-dose nitro-L-arginine administration in baboon (Papio hamadryas) pregnancy. Hennessy, A., Gillin, A.G., Duggin, G.G., Horvath, J.S., Tiller, D.J. Clin. Exp. Pharmacol. Physiol. (1999) [Pubmed]
  8. The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1. Pellizzoni, L., Baccon, J., Charroux, B., Dreyfuss, G. Curr. Biol. (2001) [Pubmed]
  9. cDNA, gene structure, and chromosomal localization of human GAR1 (CNCG3L), a homolog of the third subunit of bovine photoreceptor cGMP-gated channel. Ardell, M.D., Makhija, A.K., Oliveira, L., Miniou, P., Viegas-Péquignot, E., Pittler, S.J. Genomics (1995) [Pubmed]
  10. Stable preparation of aldose reductase isoenzymes from human placenta. Fujii, Y., Zhao, F.X., Fu, S.C., Nakai, N., Lai, C.Y. Protein Expr. Purif. (1991) [Pubmed]
  11. Mechanisms of endothelin-1-induced contraction in isolated placental veins from normal full-term and preterm pregnancies. Gallardo, V., Cruz, M.A., Miguel, P., Carrasco, G., González, C. Gen. Pharmacol. (2000) [Pubmed]
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