Protein : NR0B2

Name NR0B2
Description nuclear receptor subfamily 0, group B, member 2
Owner Public
URN urn:agi-llid:8431
Connectivity 51
Notes The protein encoded by this gene is an unusual orphan receptor that contains a putative ligand-binding domain but lacks a conventional DNA-binding domain. The gene product is a member of the nuclear hormone receptor family, a group of transcription factors regulated by small hydrophobic hormones, a subset of which do not have known ligands and are referred to as orphan nuclear receptors. The protein has been shown to interact with retinoid and thyroid hormone receptors, inhibiting their ligand-dependent transcriptional activation. In addition, interaction with estrogen receptors has been demonstrated, leading to inhibition of function. Studies suggest that the protein represses nuclear hormone receptor-mediated transactivation via two separate steps: competition with coactivators and the direct effects of its transcriptional repressor function. Acts as a novel corepressor for basic helix-loop-helix transcription factor BETA2/NeuroD. At diagnosis, methylation of SHP1 occurred more frequently in acute myeloid leukaemia than acute lymphoblastic leukaemia. Frequent methylation of SHP1, but not SOCS1, may be important in the pathogenesis, but not prognosis, of acute leukaemias. Modulation of SHP expression and/or activity in adipose tissue may therefore have significant effects on aromatase expression and estrogen production in breast adipose tissue. NR0B2 is involved in the regulation of G6Pase, CYP7A1, and PEPCK gene expression via novel mechanism of inhibition of HNF3 activity and expand the role of NR0B2 as a coregulator of other family of transcription factors in addition to nuclear receptors. PGC-1alpha mediates the ligand-dependent activation of FXR and transcription of SHP gene. Results suggest that SHP mediates recruitment of mSin3A-Swi/Snf to the CYP7A1 promoter, resulting in chromatin remodeling and gene repression. SHP affects genes involved in diverse biological pathways, e.g., several key genes involved in consecutive steps of cholesterol degradation, bile acid conjugation, transport and lipogenic pathways. SHP has a role in modulating hepatic glucocorticoid action. SHP is able to interact with LXR and to modulate its transcriptional activity. SHP mutations found in obese Danish men. SHP-1 tyrosine phosphatase is regulated in human platelets by serine phosphorylation at its C terminus. Basic helix-loop-helix (bHLH) transcription factors, the E2A proteins (E47, E12 and E2/5), activated the human but not the mouse SHP promoter. C-Jun works to activate the expression of SHP genes associated with the cascade regulation of monocytic differentiation. Crystal structure at 1.9 A resoluation of the ligand-binding domain of hLRH-1 in complex with the NR box 1 motif of human SHP, which contacts the AF-2 region of hLRH-1 using selective structural motifs. Orphan nuclear receptor small heterodimer partner promoter is regulated by sterol regulatory element binding protein-1. Orphan receptor small heterodimer partner expression is regulated by estrogen receptor alpha. Polymorphisms in the human SHP1 gene; found no rare SHP1 coding sequence variants that were exclusive to patients with lipodystrophy. Results suggest that bile acids negatively regulate the human angiotensinogen gene through the inhibitory effect of small heterodimer partner on hepatocyte nuclear factor-4. The relation between genetic variation in SHP and birth weight, adiposity, and insulin levels in three independent populations.

Microarray ID 1368376_at
d86580_at
d86745cds_s_at
d86745exon_s_at
A_43_P13041
1449854_at
97123_at
msa.2758.0_s_at
A_51_P265334
A_51_P265338
L76571
206410_at
37505_at
643_at
L76571_at
rc_w67564_s_at
A_23_P160800
OR3635
CA457
OH7312A
RA0457

GenBank ID AAB59732
AAH19540
AAQ55057
AAQ55058
AK004930
AY360323
AY360324
BC019540
L76567
NC_000070
NM_011850
NP_035980
NT_039267
Q62227
AAC41998
AAH30207
AB058644
AF044316
AI457167
AL356390
BAB68530
BC030207
CAI13562
L76571
NC_000001
NM_021969
NP_068804
NT_037485
NT_086582
Q15466
AAH88117
BAA13127
BAA13171
BC088117
D86580
D86839
NC_005104
NM_057133
NP_476474
NW_047724

Chromosome position 4 60.0 cM
1p36.1
5q36

GO ID 0004872
0004879
0003677
0006350
0006355
0008203
0000122
0003700
0003707
0003714
0005634

Alias Nr0b2
nuclear receptor subfamily 0, group B, member 2
orphan nuclear receptor SHP
short heterodimer partner
SHP-LRH1
nuclear receptor subfamily 0, group B, member II
P-SHP
Small heterodimer partner

Organism Rattus norvegicus
Mus musculus
Homo sapiens

MedLine Reference 12466851
11030331
9003453

LocusLink ID 8431
23957
117274

FunctionalClass DNA binding
ligand-dependent nuclear receptor
receptor
steroid hormone receptor
transcription corepressor
transcription factor

GO Cellular Component nucleus

Cell Localization Plasma membrane
Nucleus

Pathway PXR
PPAR all
PPARgamma
FXR
PPARalpha
LXR
CAR
ERbeta
ERalpha
LRH-1
HNF4a
NR common targets
NR common regulators
NR CYP ABCs
NR target genes

GO Biological Process cholesterol metabolism
negative regulation of transcription from RNA polymerase II promoter
regulation of transcription, DNA-dependent
transcription

Group Nuclear receptors
Nrs

Source Curated