3.1.d

Disorders of non-mitochondrial tRNA processing and aminoacyl-tRNA synthetases

  • tRNA splicing endonuclease subunit 2 deficiency (Pontocerebellar hypoplasia type 2B)
  • tRNA splicing endonuclease subunit 15 deficiency (Pontocerebellar hypoplasia type 2F)
  • tRNA splicing endonuclease subunit 34 deficiency (Pontocerebellar hypoplasia type 2C)
  • tRNA splicing endonuclease subunit 54 deficiency (Pontocerebellar hypoplasia types 2A, 4 and 5)
  • CLP1 deficiency Pontocerebellar hypoplasia type 10
  • tRNA methyltransferase 10A deficiency (Microcephaly, short stature, and impaired glucose metabolism type 1)
  • tRNA methyltransferase 1 deficiency (Autosomal recessive intellectual disability type 68)
  • DALRD3 deficiency (Early infantile epileptic encephalopathy type 86)
  • FTSJ RNA 2’-O-methyltransferase 1 deficiency (X-linked intellectual disability types 9 and 44)
  • NSUN2 deficiency (Autosomal recessive intellectual disability type 5)
  • tRNA-specific adenosine deaminase 3 deficiency (Autosomal recessive intellectual disability type 36)
  • Elongator complex protein 1 deficiency (Famillial dysautonomia)
  • Elongator complex protein 2 deficiency (Autosomal recessive intellectual disability type 58)
  • Galloway-Mowat syndrome, YRDC type
  • Galloway-Mowat syndrome, GON7 type
  • Galloway-Mowat syndrome type 2
  • Galloway-Mowat syndrome type 3
  • Galloway-Mowat syndrome type 4
  • Galloway-Mowat syndrome type 5
  • Pseudouridine synthase 3 deficiency (Autosomal recessive intellectual disability type 55)
  • Galloway-Mowat syndrome type 6
  • Alanyl-tRNA synthetase 1 deficiency (Early infantile epileptic encephalopathy type 29, recessive; axonal Charcot-Marie-Tooth disease type 2N, dominant)
  • Arginyl-tRNA synthetase 1 deficiency (Hypomyelinating leukodystrophy type 9)
  • Asparaginyl-tRNA synthetase 1 deficiency
  • Aspartyl-tRNA synthetase 1 deficiency (Hypomyelination with brainstem and spinal cord involvement and leg spasticity)
  • Cysteinyl-tRNA synthetase 1 deficiency (Microcephaly, developmental delay, and brittle hair syndrome)
  • Glutaminyl-tRNA synthetase 1 deficiency (Microcephaly, progressive, seizures, and cerebral and cerebellar atrophy)
  • Glutamyl-prolyl-tRNA synthetase 1 deficiency (Hypomyelinating leukodystrophy type 15)
  • Histidyl-tRNA synthetase 1 deficiency (Axonal Charcot-Marie-Tooth type 2W; Usher syndrome type 3B)
  • Isoleucyl-tRNA synthetase 1 deficiency (Growth retardation, impaired intellectual development, hypotonia, and hepatopathy)
  • Leucyl-tRNA synthetase 1 deficiency (Infantile liver failure syndrome type 1)
  • Lysyl-tRNA synthetase 1 deficiency (Recessive intermediate Charcot-Marie-Tooth disease type B; autosomal recessive deafness type 89)
  • Methionyl-tRNA synthetase 1 deficiency (Interstitial lung and liver disease, recessive; axonal Charcot-Marie-Tooth type 2U, dominant)
  • Phenylalanyl-tRNA synthetase subunit alpha deficiency (Rajab interstitial lung disease with brain calcifications type 2)
  • Phenylalanyl-tRNA synthetase subunit beta deficiency (Rajab interstitial lung disease with brain calcifications type 1)
  • Seryl-tRNA synthetase 1 deficiency (Neurodevelopmental disorder with microcephaly, ataxia, and seizures)
  • Threonyl-tRNA synthetase 1 deficiency (Nonphotosensitive trichothiodystrophy type 7)
  • Tryptophanyl-tRNA synthetase 1 deficiency (Distal hereditary motor neuronopathy type 9)
  • Tyrosyl-tRNA synthetase 1 deficiency (Dominant intermediate Charcot-Marie-Tooth type C)
  • Valyl-tRNA synthetase 1 deficiency (Neurodevelopmental disorder with microcephaly, seizures, and cortical atrophy)
  • AIMP1/p43 deficiency (Hypomyelinating leukodystrophy type 3)
  • AIMP2/p38 deficiency (Hypomyelinating leukodystrophy type 17)

Subnetworks

Immagini SNW 344 x 258 px 1

NOMS

Disorders of neuromodulators and other small molecules

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