Phenotype Details
Phene ID
3742
Name
Haplotype with homozygous deficiency HH5, TFB1M-related
Phene Name
Haplotype HH5
OMIA ID
1941
Species ID
9913
Characterised
Yes
Characterised Year
2016
Linked Variants
Variant IDPhenotypeGene IDDeleteriousChromosomeGenomicTranscriptProtein
963Abortion due to haplotype HH538827391819N/AN/AN/A
Linked Breeds
BreedBreed IDSpecies IDVBO Term
Holstein (black and white) (Cattle)399913http://purl.obolibrary.org/obo/VBO_0000237
Summary

[FN thanks Ekkehard Schütz for feedback on an earlier version of the text on this page]

Molecular Genetics

Schütz et al. (2016) identified the likely causal mutation as "a deletion of 138kbp, spanning position 93,233kb to 93,371kb on chromosome 9 (BTA9), harboring only dimethyl-adenosine transferase 1 (TFB1M). The deletion breakpoints are flanked by bovine long interspersed nuclear elements Bov-B (upstream) and L1ME3 (downstream), suggesting a homologous recombination/deletion event. TFB1M di-methylates adenine residues in the hairpin loop at the 3’-end of mitochondrial 12S rRNA, being essential for synthesis and function of the small ribosomal subunit of mitochondria." The causality of this deletion is reinforced by the fact that homozygous TFB1M knockout mice are not viable and suffer fetal death (Schütz et al., 2016).

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