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- UBA1: ubiquitin-like modifier activating enzyme 1
- UBE3A: ubiquitin protein ligase E3A
- UCHL1: ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase)
- UGT1A1: UDP glucuronosyltransferase 1 family, polypeptide A1
- UMOD: uromodulin
- UNC13D: unc-13 homolog D (C. elegans)
- UROD: uroporphyrinogen decarboxylase
- UROS: uroporphyrinogen III synthase
- USH1C: Usher syndrome 1C (autosomal recessive, severe)
- USH1G: Usher syndrome 1G (autosomal recessive)
- USH2A: Usher syndrome 2A (autosomal recessive, mild)
- USP9Y: ubiquitin specific peptidase 9, Y-linked
- UVSSA: UV-stimulated scaffold protein A
- VAPB: VAMP (vesicle-associated membrane protein)-associated protein B and C
- VCAN: versican
- VCP: valosin containing protein
- VHL: von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
- VLDLR: very low density lipoprotein receptor
- VPS13A: vacuolar protein sorting 13 homolog A (S. cerevisiae)
- VPS13B: vacuolar protein sorting 13 homolog B (yeast)
- VRK1: vaccinia related kinase 1
- VWF: von Willebrand factor
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