Details for: HIBADH

Gene ID: 11112

Gene Type:  Protein-coding  - A gene that serves as a template for producing a messenger RNA (mRNA) molecule, which is then translated into a functional protein.

Symbol: HIBADH

Ensembl ID: ENSG00000106049

Description: 3-hydroxyisobutyrate dehydrogenase

Selected Context(s):  Overall

Cell Significance Landscape

Contexts:

Associated with

Significant Cells

Cell Significance Index (CSI) scores for the chosen context(s)

  • hepatocyte CL0000182
    CSI 19.87
    rCSI 35.57%
    PRS 39.39
  • peripheral nervous system neuron CL2000032
    CSI 16.31
    rCSI 22.23%
    PRS 34.98
  • basal cell of epidermis CL0002187
    CSI 15.25
    rCSI 27.03%
    PRS 25.8
  • renal principal cell CL0005009
    CSI 15.06
    rCSI 39.13%
    PRS 46.29
  • CD8-positive, alpha-beta memory T cell, CD45RO-positive CL0001203
    CSI 13.4
    rCSI 16.23%
    PRS 37.96
  • GABAergic amacrine cell CL4030027
    CSI 12.71
    rCSI 43.55%
    PRS 33.72
  • duct epithelial cell CL0000068
    CSI 12.33
    rCSI 18.04%
    PRS 43.61
  • caudal ganglionic eminence derived cortical interneuron CL4023064
    CSI 12.29
    rCSI 21.71%
    PRS 25.73
  • fallopian tube secretory epithelial cell CL4030006
    CSI 11.89
    rCSI 11.45%
    PRS 41.44
  • suprabasal keratinocyte CL4033013
    CSI 11.83
    rCSI 19.31%
    PRS 21.98
  • kidney connecting tubule epithelial cell CL1000768
    CSI 11.04
    rCSI 28%
    PRS 32.31
  • retinal ganglion cell CL0000740
    CSI 10.31
    rCSI 22.77%
    PRS 30.36
  • L5 extratelencephalic projecting glutamatergic cortical neuron CL4023041
    CSI 9.92
    rCSI 35.71%
    PRS 25.6
  • colon epithelial cell CL0011108
    CSI 9.91
    rCSI 10.38%
    PRS 38.39
  • intestinal tuft cell CL0019032
    CSI 9.87
    rCSI 15.08%
    PRS 45.27
  • lung pericyte CL0009089
    CSI 9.82
    rCSI 25.91%
    PRS 47.98
  • H2 horizontal cell CL0004218
    CSI 9.76
    rCSI 48.55%
    PRS 41.01
  • H1 horizontal cell CL0004217
    CSI 9.66
    rCSI 38.26%
    PRS 44.03
  • retinal rod cell CL0000604
    CSI 9.64
    rCSI 16.98%
    PRS 39.63
  • pvalb GABAergic cortical interneuron CL4023018
    CSI 9.61
    rCSI 11.96%
    PRS 25.25
  • mesenchymal cell CL0008019
    CSI 9.5
    rCSI 24.13%
    PRS 37.52
  • melanocyte CL0000148
    CSI 9.42
    rCSI 6.98%
    PRS 35.2
  • L2/3-6 intratelencephalic projecting glutamatergic neuron CL4023040
    CSI 9.12
    rCSI 22.17%
    PRS 25.87
  • retina horizontal cell CL0000745
    CSI 9.04
    rCSI 13.78%
    PRS 37.75
  • lung macrophage CL1001603
    CSI 8.98
    rCSI 20.05%
    PRS 46.89
  • melanocyte of skin CL1000458
    CSI 8.87
    rCSI 12.09%
    PRS 21.51
  • kidney loop of Henle thin descending limb epithelial cell CL1001111
    CSI 8.41
    rCSI 11.92%
    PRS 38.14
  • chondrocyte CL0000138
    CSI 8.27
    rCSI 13.16%
    PRS 34.72
  • macroglial cell CL0000126
    CSI 8.04
    rCSI 20.66%
    PRS 44.24
  • syncytiotrophoblast cell CL0000525
    CSI 7.82
    rCSI 22.53%
    PRS 58.73
  • glycinergic amacrine cell CL4030028
    CSI 7.67
    rCSI 19.99%
    PRS 40.81
  • colonocyte CL1000347
    CSI 7.35
    rCSI 10.53%
    PRS 48.3
  • glioblast CL0000030
    CSI 7.3
    rCSI 11.64%
    PRS 35.27
  • corticothalamic-projecting glutamatergic cortical neuron CL4023013
    CSI 7.21
    rCSI 42.46%
    PRS 27.9
  • ependymal cell CL0000065
    CSI 7.13
    rCSI 14.46%
    PRS 25
  • placental villous trophoblast CL2000060
    CSI 6.91
    rCSI 10.68%
    PRS 38.93
  • chandelier pvalb GABAergic cortical interneuron CL4023036
    CSI 6.85
    rCSI 21.42%
    PRS 29.82
  • stem cell CL0000034
    CSI 6.82
    rCSI 6.58%
    PRS 32.21
  • lung neuroendocrine cell CL1000223
    CSI 6.64
    rCSI 9.82%
    PRS 45.42
  • lamp5 GABAergic cortical interneuron CL4023011
    CSI 6.41
    rCSI 10.77%
    PRS 26.65
  • secretory cell CL0000151
    CSI 6.35
    rCSI 6.63%
    PRS 41.42
  • ciliated cell CL0000064
    CSI 6.24
    rCSI 10.1%
    PRS 39.6
  • kidney interstitial alternatively activated macrophage CL1000695
    CSI 5.83
    rCSI 15.2%
    PRS 39.94
  • sst GABAergic cortical interneuron CL4023017
    CSI 5.82
    rCSI 7.51%
    PRS 27.54
  • L6b glutamatergic cortical neuron CL4023038
    CSI 5.75
    rCSI 17.98%
    PRS 27.76
  • Kupffer cell CL0000091
    CSI 5.59
    rCSI 12.78%
    PRS 40.29
  • pancreatic acinar cell CL0002064
    CSI 5.32
    rCSI 7.07%
    PRS 45.21
  • cytotoxic T cell CL0000910
    CSI 5.17
    rCSI 29.65%
    PRS 52.87
  • hepatic stellate cell CL0000632
    CSI 5.16
    rCSI 19.35%
    PRS 34.82
  • enteroendocrine cell CL0000164
    CSI 5.16
    rCSI 7.05%
    PRS 43.48
  • near-projecting glutamatergic cortical neuron CL4023012
    CSI 5.15
    rCSI 19.47%
    PRS 27.53
  • Mueller cell CL0000636
    CSI 5.08
    rCSI 11.6%
    PRS 35.07
  • sncg GABAergic cortical interneuron CL4023015
    CSI 5.01
    rCSI 8.05%
    PRS 28.67
  • renal alpha-intercalated cell CL0005011
    CSI 4.98
    rCSI 6.66%
    PRS 48.92
  • interstitial cell of Cajal CL0002088
    CSI 4.95
    rCSI 6.3%
    PRS 45.81
  • myofibroblast cell CL0000186
    CSI 4.79
    rCSI 6.63%
    PRS 46.45
  • inhibitory interneuron CL0000498
    CSI 4.7
    rCSI 10.84%
    PRS 33.57
  • pancreatic ductal cell CL0002079
    CSI 4.65
    rCSI 9.04%
    PRS 42.69
  • VIP GABAergic cortical interneuron CL4023016
    CSI 4.58
    rCSI 5.47%
    PRS 26.58
  • neural crest cell CL0011012
    CSI 4.56
    rCSI 3.61%
    PRS 29.68
  • rod bipolar cell CL0000751
    CSI 4.44
    rCSI 7.97%
    PRS 34.83
  • diffuse bipolar 6 cell CL4033032
    CSI 4.42
    rCSI 23.25%
    PRS 39.44
  • radial glial cell CL0000681
    CSI 4.42
    rCSI 6.14%
    PRS 40.46
  • early lymphoid progenitor CL0000936
    CSI 4.37
    rCSI 3.84%
    PRS 45.5
  • neuroblast (sensu Vertebrata) CL0000031
    CSI 4.3
    rCSI 5.52%
    PRS 38.89
  • naive B cell CL0000788
    CSI 4.19
    rCSI 3.59%
    PRS 50.11
  • brush cell of tracheobronchial tree CL0002075
    CSI 4.1
    rCSI 12.17%
    PRS 52.34
  • intestinal epithelial cell CL0002563
    CSI 4.04
    rCSI 4.22%
    PRS 40.59
  • multi-ciliated epithelial cell CL0005012
    CSI 4.03
    rCSI 4.02%
    PRS 35.53
  • epithelial cell of proximal tubule CL0002306
    CSI 3.97
    rCSI 9.7%
    PRS 37.48
  • erythrocyte CL0000232
    CSI 3.93
    rCSI 8.93%
    PRS 46.45
  • epithelial cell CL0000066
    CSI 3.92
    rCSI 6.03%
    PRS 43.96
  • extravillous trophoblast CL0008036
    CSI 3.85
    rCSI 4.76%
    PRS 37
  • midzonal region hepatocyte CL0019028
    CSI 3.71
    rCSI 8.72%
    PRS 50.32
  • kidney loop of Henle thin ascending limb epithelial cell CL1001107
    CSI 3.65
    rCSI 9.44%
    PRS 37.38
  • renal interstitial pericyte CL1001318
    CSI 3.65
    rCSI 10.05%
    PRS 38.03
  • fibroblast of breast CL4006000
    CSI 3.62
    rCSI 15.21%
    PRS 64.32
  • retinal bipolar neuron CL0000748
    CSI 3.57
    rCSI 6.68%
    PRS 31.6
  • ciliated epithelial cell CL0000067
    CSI 3.51
    rCSI 3.09%
    PRS 31.01
  • neuroblast (sensu Nematoda and Protostomia) CL0000338
    CSI 3.44
    rCSI 3.97%
    PRS 35.75
  • fibroblast of lung CL0002553
    CSI 3.43
    rCSI 3.19%
    PRS 40.78
  • adipocyte CL0000136
    CSI 3.42
    rCSI 4.39%
    PRS 37.21
  • kidney collecting duct intercalated cell CL1001432
    CSI 3.4
    rCSI 24.25%
    PRS 47.61
  • cerebral cortex endothelial cell CL1001602
    CSI 3.36
    rCSI 5.82%
    PRS 32.4
  • diffuse bipolar 3a cell CL4033029
    CSI 3.31
    rCSI 22.52%
    PRS 39.21
  • pro-B cell CL0000826
    CSI 3.24
    rCSI 2.68%
    PRS 41.68
  • intestine goblet cell CL0019031
    CSI 3.21
    rCSI 2.85%
    PRS 40.11
  • epithelial cell of lung CL0000082
    CSI 3.13
    rCSI 2.59%
    PRS 39.41
  • small intestine goblet cell CL1000495
    CSI 3.13
    rCSI 6.85%
    PRS 50.76
  • periportal region hepatocyte CL0019026
    CSI 3.12
    rCSI 12.14%
    PRS 50.09
  • cardiac endothelial cell CL0010008
    CSI 3.05
    rCSI 12.3%
    PRS 39.54
  • mononuclear phagocyte CL0000113
    CSI 3.02
    rCSI 6.66%
    PRS 44.63
  • mucus secreting cell CL0000319
    CSI 2.93
    rCSI 4.65%
    PRS 50.81
  • blood vessel endothelial cell CL0000071
    CSI 2.93
    rCSI 6.08%
    PRS 39.12
  • pancreatic D cell CL0000173
    CSI 2.89
    rCSI 2.84%
    PRS 43.05
  • central nervous system neuron CL2000029
    CSI 2.88
    rCSI 21.18%
    PRS 29.92
  • direct pathway medium spiny neuron CL4023026
    CSI 2.85
    rCSI 68.19%
    PRS 26.07
  • skin fibroblast CL0002620
    CSI 2.8
    rCSI 2.41%
    PRS 50.17
  • ciliated columnar cell of tracheobronchial tree CL0002145
    CSI 2.79
    rCSI 6.36%
    PRS 39.76
  • indirect pathway medium spiny neuron CL4023029
    CSI 2.79
    rCSI 67.2%
    PRS 26.95
  • mesenchymal stem cell CL0000134
    CSI 0.1
    rCSI 0.6%
    PRS 57.8%
  • medium spiny neuron CL1001474
    CSI 0.3
    rCSI 2.3%
    PRS 29.3%
  • GABAergic interneuron CL0011005
    CSI 0.4
    rCSI 5.5%
    PRS 44.3%
  • erythroblast CL0000765
    CSI 0.4
    rCSI 1.1%
    PRS 54.2%
  • intestinal crypt stem cell of colon CL0009043
    CSI 0.4
    rCSI 3.3%
    PRS 61.1%
  • eye photoreceptor cell CL0000287
    CSI 0.5
    rCSI 5.2%
    PRS 67.9%
  • S cone cell CL0003050
    CSI 0.5
    rCSI 2.0%
    PRS 38.5%
  • flat midget bipolar cell CL4033033
    CSI 0.5
    rCSI 3.5%
    PRS 37.5%
  • podocyte CL0000653
    CSI 0.5
    rCSI 2.4%
    PRS 39.5%
  • Hofbauer cell CL3000001
    CSI 0.6
    rCSI 1.1%
    PRS 50.4%
  • regular ventricular cardiac myocyte CL0002131
    CSI 0.6
    rCSI 3.8%
    PRS 34.1%
  • invaginating midget bipolar cell CL4033034
    CSI 0.6
    rCSI 3.6%
    PRS 39.5%
  • neural progenitor cell CL0011020
    CSI 0.7
    rCSI 3.0%
    PRS 35.3%
  • CD14-low, CD16-positive monocyte CL0002396
    CSI 0.7
    rCSI 0.5%
    PRS 39.4%
  • kidney connecting tubule principal cell CL4030018
    CSI 0.7
    rCSI 5.1%
    PRS 75.0%
  • diffuse bipolar 1 cell CL4033027
    CSI 0.9
    rCSI 6.7%
    PRS 37.6%
  • mammary gland epithelial cell CL0002327
    CSI 0.9
    rCSI 3.2%
    PRS 56.6%
  • stromal cell CL0000499
    CSI 0.9
    rCSI 2.6%
    PRS 42.0%
  • pulmonary artery endothelial cell CL1001568
    CSI 0.9
    rCSI 1.3%
    PRS 53.4%
  • ON parasol ganglion cell CL4033052
    CSI 1.0
    rCSI 13.6%
    PRS 34.1%
  • paneth cell of epithelium of small intestine CL1000343
    CSI 1.0
    rCSI 2.8%
    PRS 55.6%
  • glial cell CL0000125
    CSI 1.0
    rCSI 3.8%
    PRS 35.4%
  • L4 intratelencephalic projecting glutamatergic neuron CL4030063
    CSI 1.0
    rCSI 2.5%
    PRS 31.3%
  • keratocyte CL0002363
    CSI 1.1
    rCSI 2.6%
    PRS 51.3%
  • lung ciliated cell CL1000271
    CSI 1.1
    rCSI 1.3%
    PRS 31.8%
  • kidney distal convoluted tubule epithelial cell CL1000849
    CSI 1.2
    rCSI 12.2%
    PRS 42.9%
  • diffuse bipolar 2 cell CL4033028
    CSI 1.2
    rCSI 9.2%
    PRS 39.3%
  • endothelial cell of placenta CL0009092
    CSI 1.2
    rCSI 5.9%
    PRS 52.1%
  • OFF-bipolar cell CL0000750
    CSI 1.2
    rCSI 1.7%
    PRS 50.9%
  • muscle cell CL0000187
    CSI 1.2
    rCSI 2.5%
    PRS 63.2%
  • macula densa epithelial cell CL1000850
    CSI 1.2
    rCSI 17.5%
    PRS 74.1%
  • GABAergic neuron CL0000617
    CSI 1.3
    rCSI 4.2%
    PRS 30.6%
  • type B pancreatic cell CL0000169
    CSI 1.3
    rCSI 2.8%
    PRS 38.1%
  • OFFx cell CL4033036
    CSI 1.3
    rCSI 6.0%
    PRS 39.4%
  • retinal pigment epithelial cell CL0002586
    CSI 1.3
    rCSI 2.6%
    PRS 40.6%
  • hematopoietic stem cell CL0000037
    CSI 1.4
    rCSI 0.9%
    PRS 44.9%
  • CD14-positive monocyte CL0001054
    CSI 1.4
    rCSI 1.7%
    PRS 51.9%
  • dopaminergic neuron CL0000700
    CSI 1.4
    rCSI 8.0%
    PRS 28.4%
  • myoepithelial cell CL0000185
    CSI 1.4
    rCSI 3.6%
    PRS 48.8%
  • epithelial cell of proximal tubule segment 3 CL4030011
    CSI 1.4
    rCSI 11.4%
    PRS 66.1%
  • endothelial cell of pericentral hepatic sinusoid CL0019022
    CSI 1.5
    rCSI 4.5%
    PRS 53.1%
  • mesangial cell CL0000650
    CSI 1.5
    rCSI 6.0%
    PRS 53.1%
  • common myeloid progenitor CL0000049
    CSI 1.5
    rCSI 1.2%
    PRS 41.3%
  • plasmacytoid dendritic cell, human CL0001058
    CSI 1.5
    rCSI 1.1%
    PRS 42.5%
  • vascular associated smooth muscle cell CL0000359
    CSI 1.5
    rCSI 5.0%
    PRS 44.2%
  • kidney loop of Henle thick ascending limb epithelial cell CL1001106
    CSI 1.5
    rCSI 13.3%
    PRS 43.5%
  • intestinal crypt stem cell of small intestine CL0009017
    CSI 1.6
    rCSI 4.2%
    PRS 49.7%
  • adventitial cell CL0002503
    CSI 1.6
    rCSI 3.9%
    PRS 51.8%
  • epithelial cell of lower respiratory tract CL0002632
    CSI 1.6
    rCSI 1.3%
    PRS 41.1%
  • regular atrial cardiac myocyte CL0002129
    CSI 1.6
    rCSI 5.2%
    PRS 40.4%
  • diffuse bipolar 3b cell CL4033030
    CSI 1.6
    rCSI 10.9%
    PRS 40.1%
  • transit amplifying cell of colon CL0009011
    CSI 1.6
    rCSI 1.9%
    PRS 44.7%
  • intermediate monocyte CL0002393
    CSI 1.7
    rCSI 2.5%
    PRS 42.5%
  • retinal cone cell CL0000573
    CSI 1.7
    rCSI 2.7%
    PRS 32.5%
  • keratinocyte CL0000312
    CSI 1.7
    rCSI 1.4%
    PRS 45.9%
  • renal beta-intercalated cell CL0002201
    CSI 1.7
    rCSI 4.1%
    PRS 43.4%
  • pancreatic A cell CL0000171
    CSI 1.8
    rCSI 1.9%
    PRS 43.3%
  • tracheobronchial smooth muscle cell CL0019019
    CSI 1.8
    rCSI 3.2%
    PRS 49.1%
  • choroid plexus epithelial cell CL0000706
    CSI 1.8
    rCSI 3.0%
    PRS 32.5%
  • centrilobular region hepatocyte CL0019029
    CSI 1.8
    rCSI 4.7%
    PRS 49.8%
  • epicardial adipocyte CL1000309
    CSI 1.9
    rCSI 6.0%
    PRS 43.6%
  • effector memory CD8-positive, alpha-beta T cell CL0000913
    CSI 1.9
    rCSI 1.7%
    PRS 55.3%
  • megakaryocyte-erythroid progenitor cell CL0000050
    CSI 1.9
    rCSI 1.7%
    PRS 37.6%
  • intrahepatic cholangiocyte CL0002538
    CSI 2.0
    rCSI 4.7%
    PRS 57.4%
  • fibroblast of cardiac tissue CL0002548
    CSI 2.0
    rCSI 9.4%
    PRS 39.4%
  • mucous neck cell CL0000651
    CSI 2.0
    rCSI 2.9%
    PRS 54.5%
  • alveolar adventitial fibroblast CL4028006
    CSI 2.0
    rCSI 3.2%
    PRS 41.9%
  • ionocyte CL0005006
    CSI 2.1
    rCSI 2.3%
    PRS 38.8%
  • conjunctival epithelial cell CL1000432
    CSI 2.1
    rCSI 3.3%
    PRS 41.4%
  • Bergmann glial cell CL0000644
    CSI 2.1
    rCSI 2.9%
    PRS 37.9%
  • ON-bipolar cell CL0000749
    CSI 2.2
    rCSI 3.2%
    PRS 43.8%
  • endocardial cell CL0002350
    CSI 2.2
    rCSI 10.4%
    PRS 42.9%
  • microcirculation associated smooth muscle cell CL0008035
    CSI 2.2
    rCSI 6.4%
    PRS 43.4%
  • vascular leptomeningeal cell CL4023051
    CSI 2.2
    rCSI 3.9%
    PRS 33.9%
  • alveolar macrophage CL0000583
    CSI 2.2
    rCSI 3.7%
    PRS 46.1%
  • mesodermal cell CL0000222
    CSI 2.2
    rCSI 2.7%
    PRS 39.1%
  • luminal epithelial cell of mammary gland CL0002326
    CSI 2.2
    rCSI 4.1%
    PRS 56.8%
  • club cell CL0000158
    CSI 2.3
    rCSI 3.3%
    PRS 41.0%
  • amacrine cell CL0000561
    CSI 2.3
    rCSI 6.7%
    PRS 33.2%
  • lung secretory cell CL1000272
    CSI 2.3
    rCSI 5.8%
    PRS 39.0%
  • OFF midget ganglion cell CL4033047
    CSI 2.3
    rCSI 47.6%
    PRS 35.5%
  • alveolar type 1 fibroblast cell CL4028004
    CSI 2.4
    rCSI 2.6%
    PRS 44.6%
  • differentiation-committed oligodendrocyte precursor CL4023059
    CSI 2.4
    rCSI 4.4%
    PRS 35.2%
  • parietal epithelial cell CL1000452
    CSI 2.4
    rCSI 6.5%
    PRS 34.6%
  • cardiac neuron CL0010022
    CSI 2.5
    rCSI 7.9%
    PRS 38.1%
  • ON midget ganglion cell CL4033046
    CSI 2.5
    rCSI 50.6%
    PRS 34.3%
  • astrocyte of the cerebral cortex CL0002605
    CSI 2.5
    rCSI 5.6%
    PRS 27.3%
  • innate lymphoid cell CL0001065
    CSI 2.5
    rCSI 5.2%
    PRS 47.9%
  • BEST4+ enteroycte CL4030026
    CSI 2.6
    rCSI 3.2%
    PRS 43.5%
  • interneuron CL0000099
    CSI 2.6
    rCSI 5.2%
    PRS 31.6%
  • cardiac muscle cell CL0000746
    CSI 2.6
    rCSI 3.7%
    PRS 33.1%
  • Schwann cell CL0002573
    CSI 2.6
    rCSI 7.4%
    PRS 41.0%
  • cerebellar granule cell CL0001031
    CSI 2.6
    rCSI 3.8%
    PRS 37.5%
  • helper T cell CL0000912
    CSI 2.7
    rCSI 3.8%
    PRS 49.9%
  • enteric smooth muscle cell CL0002504
    CSI 2.7
    rCSI 3.8%
    PRS 43.6%
  • contractile cell CL0000183
    CSI 2.7
    rCSI 7.9%
    PRS 40.1%
  • indirect pathway medium spiny neuron CL4023029
    CSI 2.8
    rCSI 67.2%
    PRS 27.0%
  • ciliated columnar cell of tracheobronchial tree CL0002145
    CSI 2.8
    rCSI 6.4%
    PRS 39.8%
  • skin fibroblast CL0002620
    CSI 2.8
    rCSI 2.4%
    PRS 50.2%
  • direct pathway medium spiny neuron CL4023026
    CSI 2.9
    rCSI 68.2%
    PRS 26.1%

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.
Network Configuration

Explore relationships of the current gene. Select an Interaction Source: 'ONTOLOGY' for shared pathways (GO/Reactome) or 'STRING' for protein-protein interactions. Further refine by selecting context genes and comparing Cell Significance Index (CSI) scores between baseline and target cell types and their specific contexts.

Comma-separated if multiple.
Comma-separated if multiple.

Legend:
  • Query Gene
  • Node Color (Target Cell CSI, relative to current network):
    • Very High
    • High
    • Medium
    • Low
    • Very Low
    • CSI N/A
  • Node Size: Proportional to Target Cell CSI magnitude
  • STRING PPI Edge
  • Shared Pathway Edge (ONTOLOGY)

Loading network (please wait)...

Other Information

This section provides additional information about the gene, including a description generated by an AI language model and details about associated proteins.

## Summary [HIBADH](/details-gene/11112) (3-hydroxyisobutyrate dehydrogenase) is a protein-coding gene located on chromosome 7p15.2. It encodes a crucial mitochondrial enzyme that catalyzes a key step in the catabolic pathway of the branched-chain amino acid valine. Functionally, it is responsible for the NAD+-dependent oxidation of 3-hydroxyisobutyrate to methylmalonate semialdehyde. Consistent with its role in core metabolism, [HIBADH](/details-gene/11112) shows significant expression in metabolically active tissues. **Overall**, its highest significance is observed in [hepatocytes](/details-cell/CL0000182), reflecting the liver's central role in amino acid processing. Notably, it is also highly expressed in various neuronal and epithelial cell types, suggesting a broad importance in cellular energy homeostasis. Mutations in this gene are associated with the inborn error of metabolism known as 3-hydroxyisobutyric aciduria [Link](https://doi.org/10.1016/j.ymgme.2005.09.019). ## Cellular Roles and Expression Landscape The expression profile of [HIBADH](/details-gene/11112) underscores its fundamental role in cellular metabolism, with a widespread but distinct pattern across multiple lineages. **Overall**, the gene's highest significance is found in cell types with high energy demands and active metabolic functions. The top-ranked cell type is the [hepatocyte](/details-cell/CL0000182) (CSI: 19.87), which is the primary site for branched-chain amino acid catabolism in the body. Beyond the liver, [HIBADH](/details-gene/11112) is also a significant gene in various components of the nervous system, including [peripheral nervous system neuron](/details-cell/CL2000032) (CSI: 16.31), [GABAergic amacrine cell](/details-cell/CL4030027) (CSI: 12.71), and [retinal ganglion cell](/details-cell/CL0000740) (CSI: 10.31). This suggests that local valine catabolism may be an important energy source for neuronal maintenance and function. Furthermore, a distinct signature of [HIBADH](/details-gene/11112) expression is observed in various epithelial and barrier tissues. These include [basal cell of epidermis](/details-cell/CL0002187) (CSI: 15.25), [renal principal cell](/details-cell/CL0005009) (CSI: 15.06), [duct epithelial cell](/details-cell/CL0000068) (CSI: 12.33), and [colon epithelial cell](/details-cell/CL0011108) (CSI: 9.91). This pattern is consistent with the high metabolic turnover required for tissue homeostasis, proliferation, and maintenance of physiological barriers in these locations. A more moderate but notable expression is also seen in specific immune subsets, such as the [CD8-positive, alpha-beta memory T cell, CD45RO-positive](/details-cell/CL0001203) (CSI: 13.40), indicating a potential role in supporting the metabolic requirements of long-lived lymphocytes. ## Pathways and Molecular Function Functional annotation data provide a clear mechanistic basis for the observed expression patterns of [HIBADH](/details-gene/11112). The gene product is a mitochondrial matrix enzyme ([GO:0005759](https://www.ebi.ac.uk/QuickGO/term/GO:0005759)) with a primary molecular function of 3-hydroxyisobutyrate dehydrogenase activity ([GO:0008442](https://www.ebi.ac.uk/QuickGO/term/GO:0008442)). This activity is essential for the **Valine catabolic process** ([GO:0006574](https://www.ebi.ac.uk/QuickGO/term/GO:0006574)). At the pathway level, [HIBADH](/details-gene/11112) is a component of the **Branched-chain amino acid catabolism** pathway ([R-HSA-70895](https://reactome.org/content/detail/R-HSA-70895)), which is part of the broader **Metabolism of amino acids and derivatives** ([R-HSA-71291](https://reactome.org/content/detail/R-HSA-71291)). The enzyme's dependence on NAD+ or NADP+ as a cofactor is supported by annotations for **Nad binding** ([GO:0051287](https://www.ebi.ac.uk/QuickGO/term/GO:0051287)) and **Nadp binding** ([GO:0050661](https://www.ebi.ac.uk/QuickGO/term/GO:0050661)). This functional profile firmly establishes [HIBADH](/details-gene/11112) as a specialized enzyme for amino acid metabolism, explaining its high expression in cells like [hepatocytes](/details-cell/CL0000182) that are central to processing circulating nutrients. ## Research Directions The widespread expression of [HIBADH](/details-gene/11112) in metabolically active but diverse cell types beyond the liver suggests un-explored, tissue-specific roles for local valine catabolism. ### Proposed Hypotheses: 1. **Role in Neuro-Metabolic Coupling:** The high expression of [HIBADH](/details-gene/11112) in multiple distinct neuronal subtypes ([peripheral nervous system neuron](/details-cell/CL2000032), [GABAergic amacrine cell](/details-cell/CL4030027), [retinal ganglion cell](/details-cell/CL0000740)) suggests that localized valine catabolism serves as a critical on-site energy source to support synaptic transmission and neuronal integrity. Impaired [HIBADH](/details-gene/11112) function in these cells may lead to localized energy deficits or toxic metabolite accumulation, contributing to the pathophysiology of neurodegenerative disorders independent of systemic metabolic dysfunction. 2. **Function in Epithelial Barrier Integrity:** The significant expression in epithelial cells of the skin ([basal cell of epidermis](/details-cell/CL0002187)), kidney ([renal principal cell](/details-cell/CL0005009)), and colon ([colon epithelial cell](/details-cell/CL0011108)) indicates that [HIBADH](/details-gene/11112)-mediated metabolism is necessary to fuel the high energy demands of tissue turnover, ion transport, and barrier maintenance. Downregulation of [HIBADH](/details-gene/11112) in these tissues under conditions of stress or injury could impair wound healing or compromise barrier function, increasing susceptibility to infection or inflammation. ### Key Experimental Approach: To test the hypothesis regarding its role in neuro-metabolic coupling, a cell-type-specific investigation is warranted. A key experiment would involve generating a conditional knockout mouse model using the Cre-lox system to specifically delete [HIBADH](/details-gene/11112) in a defined neuronal population (e.g., using a *Syn1*-Cre driver for pan-neuronal deletion or a more specific driver for a subtype like *Gad2*-Cre for GABAergic neurons). These conditional knockout mice and their littermate controls could then be subjected to metabolic challenges (e.g., ketogenic diet) or aging studies. The impact of [HIBADH](/details-gene/11112) loss would be assessed using a combination of behavioral tests for cognitive and motor function, *in vivo* two-photon imaging to monitor neuronal activity, and ex vivo metabolomics on isolated brain regions to quantify the accumulation of 3-hydroxyisobutyrate and its effect on TCA cycle intermediates. ### Therapeutic Potential: As [HIBADH](/details-gene/11112) deficiency causes a known inborn error of metabolism, therapeutic strategies would focus on **restoration of function or activation**, rather than inhibition. For patients with 3-hydroxyisobutyric aciduria, potential avenues include AAV-mediated gene therapy aimed at restoring enzymatic function, particularly in the liver and central nervous system. An alternative approach could involve the development of small-molecule pharmacological chaperones designed to stabilize partially functional mutant [HIBADH](/details-gene/11112) protein, thereby increasing residual enzyme activity. Inhibition of [HIBADH](/details-gene/11112) would be therapeutically detrimental as it would phenocopy the disease state.

Genular Protein ID: 622721413

Symbol: 3HIDH_HUMAN

Name: 3-hydroxyisobutyrate dehydrogenase, mitochondrial

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 12853948

Title: The DNA sequence of human chromosome 7.

PubMed ID: 12853948

DOI: 10.1038/nature01782

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 8313870

Title: Human liver protein map: update 1993.

PubMed ID: 8313870

DOI: 10.1002/elps.11501401181

PubMed ID: 16466957

Title: Clinical, biochemical, and molecular findings in three patients with 3-hydroxyisobutyric aciduria.

PubMed ID: 16466957

DOI: 10.1016/j.ymgme.2005.09.019

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

Sequence Information:

  • Length: 336
  • Mass: 35329
  • Checksum: DA3128774A91AF48
  • Sequence:
  • MAASLRLLGA ASGLRYWSRR LRPAAGSFAA VCSRSVASKT PVGFIGLGNM GNPMAKNLMK 
    HGYPLIIYDV FPDACKEFQD AGEQVVSSPA DVAEKADRII TMLPTSINAI EAYSGANGIL 
    KKVKKGSLLI DSSTIDPAVS KELAKEVEKM GAVFMDAPVS GGVGAARSGN LTFMVGGVED 
    EFAAAQELLG CMGSNVVYCG AVGTGQAAKI CNNMLLAISM IGTAEAMNLG IRLGLDPKLL 
    AKILNMSSGR CWSSDTYNPV PGVMDGVPSA NNYQGGFGTT LMAKDLGLAQ DSATSTKSPI 
    LLGSLAHQIY RMMCAKGYSK KDFSSVFQFL REEETF