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PMID:21048919

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Citation

Al-Shami, A, Jhaver, KG, Vogel, P, Wilkins, C, Humphries, J, Davis, JJ, Xu, N, Potter, DG, Gerhardt, B, Mullinax, R, Shirley, CR, Anderson, SJ and Oravecz, T (2010) Regulators of the proteasome pathway, Uch37 and Rpn13, play distinct roles in mouse development. PLoS ONE 5:e13654

Abstract

Rpn13 is a novel mammalian proteasomal receptor that has recently been identified as an amplification target in ovarian cancer. It can interact with ubiquitin and activate the deubiquitinating enzyme Uch37 at the 26S proteasome. Since neither Rpn13 nor Uch37 is an integral proteasomal subunit, we explored whether either protein is essential for mammalian development and survival. Deletion of Uch37 resulted in prenatal lethality in mice associated with severe defect in embryonic brain development. In contrast, the majority of Rpn13-deficient mice survived to adulthood, although they were smaller at birth and fewer in number than wild-type littermates. Absence of Rpn13 produced tissue-specific effects on proteasomal function: increased proteasome activity in adrenal gland and lymphoid organs, and decreased activity in testes and brain. Adult Rpn13(-/-) mice reached normal body weight but had increased body fat content and were infertile due to defective gametogenesis. Additionally, Rpn13(-/-) mice showed increased T-cell numbers, resembling growth hormone-mediated effects. Indeed, serum growth hormone and follicular stimulating hormone levels were significantly increased in Rpn13(-/-) mice, while growth hormone receptor expression was reduced in the testes. In conclusion, this is the first report characterizing the physiological roles of Uch37 and Rpn13 in murine development and implicating a non-ATPase proteasomal protein, Rpn13, in the process of gametogenesis.

Links

PubMed PMC2965108 Online version:10.1371/journal.pone.0013654

Keywords

Animals; Base Sequence; Blotting, Western; DNA Primers; Flow Cytometry; Membrane Glycoproteins/physiology; Mice; Mice, Mutant Strains; Nuclear Magnetic Resonance, Biomolecular; Oogenesis/physiology; Proteasome Endopeptidase Complex/metabolism; RNA, Messenger/genetics; Spermatogenesis/physiology

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

MOUSE:UCHL5

involved_in

GO:0048853: forebrain morphogenesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:UCHL5

involved_in

GO:0030901: midbrain development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:UCHL5

involved_in

GO:0021670: lateral ventricle development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0042699: follicle-stimulating hormone signaling pathway

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0048477: oogenesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0048538: thymus development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0072520: seminiferous tubule development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0060612: adipose tissue development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0060399: positive regulation of growth hormone receptor signaling pathway

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0033081: regulation of T cell differentiation in thymus

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0007286: spermatid development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0001541: ovarian follicle development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:ADRM1

involved_in

GO:0060009: Sertoli cell development

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete


See also

References

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