Details for: HIBCH

Gene ID: 26275

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: HIBCH

Ensembl ID: ENSG00000198130

Description: 3-hydroxyisobutyryl-CoA hydrolase

Selected Context(s):  Overall

Cell Significance Landscape

Contexts:

Associated with

Significant Cells

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

  • epithelial cell of lung CL0000082
    CSI 15.74
    rCSI 13.05%
    PRS 42.94
  • epithelial cell of proximal tubule CL0002306
    CSI 13.74
    rCSI 33.55%
    PRS 40.56
  • double negative thymocyte CL0002489
    CSI 12.94
    rCSI 9%
    PRS 52.92
  • near-projecting glutamatergic cortical neuron CL4023012
    CSI 11.68
    rCSI 44.15%
    PRS 30.15
  • enteroendocrine cell CL0000164
    CSI 10.39
    rCSI 14.2%
    PRS 47.07
  • pvalb GABAergic cortical interneuron CL4023018
    CSI 9.93
    rCSI 12.35%
    PRS 27.76
  • colonocyte CL1000347
    CSI 9
    rCSI 12.89%
    PRS 51.7
  • central nervous system neuron CL2000029
    CSI 8.97
    rCSI 65.94%
    PRS 32.82
  • S cone cell CL0003050
    CSI 8.79
    rCSI 38.63%
    PRS 42.24
  • intermediate monocyte CL0002393
    CSI 8.2
    rCSI 12.38%
    PRS 46.45
  • endocardial cell CL0002350
    CSI 8.19
    rCSI 39.23%
    PRS 45.93
  • L5 extratelencephalic projecting glutamatergic cortical neuron CL4023041
    CSI 8.12
    rCSI 29.23%
    PRS 28.15
  • adipocyte CL0000136
    CSI 7.52
    rCSI 9.65%
    PRS 40.08
  • sst GABAergic cortical interneuron CL4023017
    CSI 7.3
    rCSI 9.41%
    PRS 30.1
  • plasmablast CL0000980
    CSI 7.3
    rCSI 5.74%
    PRS 50.8
  • epithelial cell of proximal tubule segment 3 CL4030011
    CSI 6.79
    rCSI 53.98%
    PRS 67.47
  • L6b glutamatergic cortical neuron CL4023038
    CSI 6.71
    rCSI 20.96%
    PRS 30.39
  • type L enteroendocrine cell CL0002279
    CSI 6.63
    rCSI 12.44%
    PRS 63.85
  • differentiation-committed oligodendrocyte precursor CL4023059
    CSI 6.37
    rCSI 11.57%
    PRS 38.1
  • enterocyte CL0000584
    CSI 6.33
    rCSI 10.2%
    PRS 53.26
  • pulmonary artery endothelial cell CL1001568
    CSI 6.28
    rCSI 8.55%
    PRS 57.19
  • central nervous system macrophage CL0000878
    CSI 6.1
    rCSI 20.22%
    PRS 46.4
  • GABAergic amacrine cell CL4030027
    CSI 6.06
    rCSI 20.76%
    PRS 36.27
  • immature B cell CL0000816
    CSI 6.01
    rCSI 4.46%
    PRS 57.52
  • neuroblast (sensu Vertebrata) CL0000031
    CSI 5.9
    rCSI 7.57%
    PRS 42.49
  • ciliated columnar cell of tracheobronchial tree CL0002145
    CSI 5.62
    rCSI 12.81%
    PRS 43.03
  • alveolar type 1 fibroblast cell CL4028004
    CSI 5.61
    rCSI 6.14%
    PRS 48.3
  • CD4-positive, CD25-positive, alpha-beta regulatory T cell CL0000792
    CSI 4.94
    rCSI 4.86%
    PRS 59.72
  • endothelial cell of pericentral hepatic sinusoid CL0019022
    CSI 4.88
    rCSI 15.04%
    PRS 56.27
  • hepatocyte CL0000182
    CSI 4.86
    rCSI 8.69%
    PRS 42.83
  • corticothalamic-projecting glutamatergic cortical neuron CL4023013
    CSI 4.6
    rCSI 27.06%
    PRS 30.64
  • endothelial cell of vascular tree CL0002139
    CSI 4.58
    rCSI 25.02%
    PRS 47.35
  • retinal ganglion cell CL0000740
    CSI 4.53
    rCSI 10.01%
    PRS 33.18
  • interneuron CL0000099
    CSI 4.37
    rCSI 8.78%
    PRS 34.62
  • retinal cone cell CL0000573
    CSI 4.35
    rCSI 7.01%
    PRS 35.45
  • oligodendrocyte precursor cell CL0002453
    CSI 4.32
    rCSI 9.51%
    PRS 30.43
  • club cell CL0000158
    CSI 4.31
    rCSI 6.32%
    PRS 44
  • glioblast CL0000030
    CSI 3.91
    rCSI 6.24%
    PRS 38.48
  • megakaryocyte-erythroid progenitor cell CL0000050
    CSI 3.9
    rCSI 3.53%
    PRS 41.17
  • intestinal epithelial cell CL0002563
    CSI 3.88
    rCSI 4.06%
    PRS 43.96
  • kidney loop of Henle thin ascending limb epithelial cell CL1001107
    CSI 3.73
    rCSI 9.64%
    PRS 40.78
  • central memory CD4-positive, alpha-beta T cell CL0000904
    CSI 3.67
    rCSI 2.17%
    PRS 59.43
  • fallopian tube secretory epithelial cell CL4030006
    CSI 3.65
    rCSI 3.51%
    PRS 44.91
  • enteroglial cell CL4040002
    CSI 3.62
    rCSI 19.03%
    PRS 52.94
  • rod bipolar cell CL0000751
    CSI 3.57
    rCSI 6.42%
    PRS 38.02
  • alpha-beta T cell CL0000789
    CSI 3.54
    rCSI 4.14%
    PRS 60
  • renal interstitial pericyte CL1001318
    CSI 3.53
    rCSI 9.73%
    PRS 41.22
  • Mueller cell CL0000636
    CSI 3.52
    rCSI 8.04%
    PRS 38.07
  • neural crest cell CL0011012
    CSI 3.51
    rCSI 2.77%
    PRS 32.57
  • renal principal cell CL0005009
    CSI 3.41
    rCSI 8.85%
    PRS 49.44
  • pulmonary alveolar type 1 cell CL0002062
    CSI 3.32
    rCSI 19.15%
    PRS 46.33
  • enteric smooth muscle cell CL0002504
    CSI 3.28
    rCSI 4.68%
    PRS 47.07
  • neuroblast (sensu Nematoda and Protostomia) CL0000338
    CSI 3.25
    rCSI 3.75%
    PRS 38.94
  • kidney connecting tubule epithelial cell CL1000768
    CSI 3.25
    rCSI 8.24%
    PRS 35.33
  • naive B cell CL0000788
    CSI 3.24
    rCSI 2.78%
    PRS 53.45
  • mucosal invariant T cell CL0000940
    CSI 3.22
    rCSI 2.6%
    PRS 55.3
  • VIP GABAergic cortical interneuron CL4023016
    CSI 3.13
    rCSI 3.74%
    PRS 29.21
  • astrocyte of the cerebral cortex CL0002605
    CSI 3.1
    rCSI 6.95%
    PRS 29.91
  • kidney loop of Henle thin descending limb epithelial cell CL1001111
    CSI 3.05
    rCSI 4.32%
    PRS 41.53
  • radial glial cell CL0000681
    CSI 3
    rCSI 4.17%
    PRS 43.89
  • fibroblast of cardiac tissue CL0002548
    CSI 3
    rCSI 14.36%
    PRS 42.94
  • ependymal cell CL0000065
    CSI 2.97
    rCSI 6.02%
    PRS 27.58
  • hematopoietic stem cell CL0000037
    CSI 2.95
    rCSI 1.96%
    PRS 48.11
  • direct pathway medium spiny neuron CL4023026
    CSI 2.94
    rCSI 70.46%
    PRS 28.7
  • ON-bipolar cell CL0000749
    CSI 2.94
    rCSI 4.37%
    PRS 46.97
  • hematopoietic precursor cell CL0008001
    CSI 2.94
    rCSI 3.02%
    PRS 62.09
  • hepatic stellate cell CL0000632
    CSI 2.93
    rCSI 10.98%
    PRS 37.74
  • type EC enteroendocrine cell CL0000577
    CSI 2.91
    rCSI 10.33%
    PRS 57.03
  • effector CD8-positive, alpha-beta T cell CL0001050
    CSI 2.9
    rCSI 2.21%
    PRS 55.72
  • indirect pathway medium spiny neuron CL4023029
    CSI 2.9
    rCSI 69.88%
    PRS 29.58
  • early lymphoid progenitor CL0000936
    CSI 2.88
    rCSI 2.53%
    PRS 49.32
  • neuron CL0000540
    CSI 2.8
    rCSI 7.46%
    PRS 36.92
  • precursor B cell CL0000817
    CSI 2.78
    rCSI 2.43%
    PRS 54.16
  • goblet cell CL0000160
    CSI 2.73
    rCSI 2.58%
    PRS 45.36
  • myofibroblast cell CL0000186
    CSI 2.67
    rCSI 3.7%
    PRS 49.3
  • retinal bipolar neuron CL0000748
    CSI 2.67
    rCSI 5%
    PRS 34.6
  • vascular leptomeningeal cell CL4023051
    CSI 2.67
    rCSI 4.68%
    PRS 37.05
  • melanocyte CL0000148
    CSI 2.66
    rCSI 1.97%
    PRS 38.18
  • midzonal region hepatocyte CL0019028
    CSI 2.61
    rCSI 6.12%
    PRS 53.46
  • interstitial cell of Cajal CL0002088
    CSI 2.58
    rCSI 3.28%
    PRS 49.65
  • pro-B cell CL0000826
    CSI 2.52
    rCSI 2.08%
    PRS 45.37
  • ON midget ganglion cell CL4033046
    CSI 2.46
    rCSI 50.13%
    PRS 37.36
  • mucus secreting cell CL0000319
    CSI 2.45
    rCSI 3.89%
    PRS 55.04
  • mature T cell CL0002419
    CSI 2.44
    rCSI 1.9%
    PRS 61.19
  • blood vessel endothelial cell CL0000071
    CSI 2.44
    rCSI 5.07%
    PRS 42.38
  • OFF midget ganglion cell CL4033047
    CSI 2.43
    rCSI 49.49%
    PRS 38.37
  • enteroendocrine cell of small intestine CL0009006
    CSI 2.4
    rCSI 5.29%
    PRS 58.85
  • transit amplifying cell of colon CL0009011
    CSI 2.36
    rCSI 2.77%
    PRS 48.13
  • cerebellar granule cell CL0001031
    CSI 2.33
    rCSI 3.43%
    PRS 40.49
  • ciliated cell CL0000064
    CSI 2.33
    rCSI 3.77%
    PRS 42.9
  • parietal epithelial cell CL1000452
    CSI 2.31
    rCSI 6.17%
    PRS 37.58
  • renal alpha-intercalated cell CL0005011
    CSI 2.28
    rCSI 3.05%
    PRS 52.85
  • dopaminergic neuron CL0000700
    CSI 2.26
    rCSI 12.75%
    PRS 31.21
  • epithelial cell CL0000066
    CSI 2.24
    rCSI 3.45%
    PRS 46.59
  • intestine goblet cell CL0019031
    CSI 2.24
    rCSI 1.99%
    PRS 43.53
  • brush cell of tracheobronchial tree CL0002075
    CSI 2.23
    rCSI 6.62%
    PRS 56.07
  • podocyte CL0000653
    CSI 2.21
    rCSI 9.83%
    PRS 43.15
  • regular atrial cardiac myocyte CL0002129
    CSI 2.18
    rCSI 7.02%
    PRS 43.79
  • fibroblast of lung CL0002553
    CSI 2.17
    rCSI 2.02%
    PRS 44.25
  • paneth cell of epithelium of small intestine CL1000343
    CSI 2.15
    rCSI 6.03%
    PRS 58.85
  • respiratory goblet cell CL0002370
    CSI 0.2
    rCSI 2.2%
    PRS 64.9%
  • cytotoxic T cell CL0000910
    CSI 0.3
    rCSI 1.5%
    PRS 56.5%
  • enterocyte of epithelium of small intestine CL1000334
    CSI 0.3
    rCSI 4.2%
    PRS 70.0%
  • regular ventricular cardiac myocyte CL0002131
    CSI 0.3
    rCSI 1.8%
    PRS 37.2%
  • epithelial cell of esophagus CL0002252
    CSI 0.3
    rCSI 3.2%
    PRS 71.1%
  • medium spiny neuron CL1001474
    CSI 0.3
    rCSI 2.8%
    PRS 32.4%
  • erythroid progenitor cell CL0000038
    CSI 0.4
    rCSI 2.2%
    PRS 55.0%
  • H2 horizontal cell CL0004218
    CSI 0.4
    rCSI 2.0%
    PRS 44.1%
  • vein endothelial cell of respiratory system CL4033008
    CSI 0.4
    rCSI 2.8%
    PRS 62.6%
  • mesenchymal stem cell CL0000134
    CSI 0.4
    rCSI 4.7%
    PRS 60.6%
  • fibroblast of breast CL4006000
    CSI 0.4
    rCSI 1.9%
    PRS 65.9%
  • basal cell of epidermis CL0002187
    CSI 0.5
    rCSI 0.8%
    PRS 27.5%
  • stromal cell of ovary CL0002132
    CSI 0.5
    rCSI 1.4%
    PRS 59.9%
  • paneth cell CL0000510
    CSI 0.5
    rCSI 0.8%
    PRS 61.9%
  • retina horizontal cell CL0000745
    CSI 0.5
    rCSI 0.8%
    PRS 41.2%
  • cerebral cortex pyramidal neuron CL4023111
    CSI 0.5
    rCSI 3.3%
    PRS 66.3%
  • antibody secreting cell CL0000946
    CSI 0.6
    rCSI 2.6%
    PRS 85.3%
  • cerebellar neuron CL1001611
    CSI 0.6
    rCSI 5.2%
    PRS 33.5%
  • H1 horizontal cell CL0004217
    CSI 0.6
    rCSI 2.4%
    PRS 47.0%
  • endothelial cell of placenta CL0009092
    CSI 0.6
    rCSI 3.0%
    PRS 56.2%
  • deuterosomal cell CL4033044
    CSI 0.6
    rCSI 2.1%
    PRS 53.7%
  • cardiac blood vessel endothelial cell CL0010006
    CSI 0.7
    rCSI 4.8%
    PRS 38.6%
  • enterocyte of epithelium of large intestine CL0002071
    CSI 0.7
    rCSI 3.7%
    PRS 59.0%
  • chandelier pvalb GABAergic cortical interneuron CL4023036
    CSI 0.7
    rCSI 2.2%
    PRS 32.7%
  • IgG plasma cell CL0000985
    CSI 0.7
    rCSI 0.9%
    PRS 63.6%
  • intestinal crypt stem cell of colon CL0009043
    CSI 0.7
    rCSI 5.5%
    PRS 64.6%
  • endothelial cell of arteriole CL1000412
    CSI 0.7
    rCSI 4.1%
    PRS 67.4%
  • kidney distal convoluted tubule epithelial cell CL1000849
    CSI 0.7
    rCSI 7.9%
    PRS 45.7%
  • amacrine cell CL0000561
    CSI 0.8
    rCSI 2.2%
    PRS 36.1%
  • placental villous trophoblast CL2000060
    CSI 0.8
    rCSI 1.2%
    PRS 42.4%
  • lung ciliated cell CL1000271
    CSI 0.8
    rCSI 0.9%
    PRS 34.9%
  • cholangiocyte CL1000488
    CSI 0.8
    rCSI 4.8%
    PRS 58.5%
  • mesothelial cell CL0000077
    CSI 0.8
    rCSI 3.2%
    PRS 22.1%
  • basal-myoepithelial cell of mammary gland CL0002324
    CSI 0.8
    rCSI 1.6%
    PRS 67.4%
  • GABAergic interneuron CL0011005
    CSI 0.9
    rCSI 13.6%
    PRS 48.7%
  • transit amplifying cell of small intestine CL0009012
    CSI 0.9
    rCSI 3.8%
    PRS 63.7%
  • duct epithelial cell CL0000068
    CSI 0.9
    rCSI 1.3%
    PRS 47.4%
  • caudal ganglionic eminence derived cortical interneuron CL4023064
    CSI 0.9
    rCSI 1.6%
    PRS 28.3%
  • Hofbauer cell CL3000001
    CSI 0.9
    rCSI 1.7%
    PRS 54.6%
  • CD14-low, CD16-positive monocyte CL0002396
    CSI 0.9
    rCSI 0.7%
    PRS 43.3%
  • keratocyte CL0002363
    CSI 0.9
    rCSI 2.2%
    PRS 54.6%
  • OFFx cell CL4033036
    CSI 0.9
    rCSI 4.4%
    PRS 42.2%
  • type B pancreatic cell CL0000169
    CSI 0.9
    rCSI 2.1%
    PRS 41.8%
  • colon goblet cell CL0009039
    CSI 0.9
    rCSI 2.2%
    PRS 56.6%
  • kidney epithelial cell CL0002518
    CSI 1.0
    rCSI 1.8%
    PRS 67.7%
  • stromal cell CL0000499
    CSI 1.0
    rCSI 2.7%
    PRS 44.6%
  • tuft cell of colon CL0009041
    CSI 1.0
    rCSI 2.2%
    PRS 62.1%
  • L5/6 near-projecting glutamatergic neuron CL4030067
    CSI 1.0
    rCSI 3.2%
    PRS 33.6%
  • L2/3 intratelencephalic projecting glutamatergic neuron CL4030059
    CSI 1.0
    rCSI 2.2%
    PRS 35.0%
  • GABAergic neuron CL0000617
    CSI 1.0
    rCSI 3.4%
    PRS 33.2%
  • CD8-positive, CD28-negative, alpha-beta regulatory T cell CL0000920
    CSI 1.0
    rCSI 2.0%
    PRS 62.1%
  • ON parasol ganglion cell CL4033052
    CSI 1.0
    rCSI 14.4%
    PRS 37.0%
  • glutamatergic neuron CL0000679
    CSI 1.1
    rCSI 2.2%
    PRS 39.0%
  • CD14-positive monocyte CL0001054
    CSI 1.1
    rCSI 1.3%
    PRS 55.8%
  • mesodermal cell CL0000222
    CSI 1.1
    rCSI 1.3%
    PRS 42.5%
  • colon epithelial cell CL0011108
    CSI 1.1
    rCSI 1.2%
    PRS 41.7%
  • forebrain radial glial cell CL0013000
    CSI 1.2
    rCSI 3.7%
    PRS 51.9%
  • small intestine goblet cell CL1000495
    CSI 1.2
    rCSI 2.6%
    PRS 54.3%
  • cerebral cortex neuron CL0010012
    CSI 1.2
    rCSI 4.9%
    PRS 41.9%
  • neural progenitor cell CL0011020
    CSI 1.2
    rCSI 5.3%
    PRS 38.1%
  • extravillous trophoblast CL0008036
    CSI 1.2
    rCSI 1.5%
    PRS 40.4%
  • mesenchymal cell CL0008019
    CSI 1.2
    rCSI 3.1%
    PRS 40.4%
  • L4 intratelencephalic projecting glutamatergic neuron CL4030063
    CSI 1.2
    rCSI 2.9%
    PRS 34.2%
  • eye photoreceptor cell CL0000287
    CSI 1.2
    rCSI 13.8%
    PRS 69.6%
  • L2/3-6 intratelencephalic projecting glutamatergic neuron CL4023040
    CSI 1.2
    rCSI 3.0%
    PRS 28.4%
  • retinal blood vessel endothelial cell CL0002585
    CSI 1.2
    rCSI 2.0%
    PRS 48.2%
  • retinal pigment epithelial cell CL0002586
    CSI 1.2
    rCSI 2.5%
    PRS 43.8%
  • keratinocyte CL0000312
    CSI 1.2
    rCSI 1.0%
    PRS 49.4%
  • cardiac endothelial cell CL0010008
    CSI 1.3
    rCSI 5.0%
    PRS 42.9%
  • pancreatic ductal cell CL0002079
    CSI 1.3
    rCSI 2.4%
    PRS 46.5%
  • tendon cell CL0000388
    CSI 1.3
    rCSI 3.3%
    PRS 67.5%
  • pancreatic A cell CL0000171
    CSI 1.3
    rCSI 1.3%
    PRS 47.2%
  • effector CD4-positive, alpha-beta T cell CL0001044
    CSI 1.3
    rCSI 3.7%
    PRS 62.0%
  • common myeloid progenitor CL0000049
    CSI 1.3
    rCSI 1.0%
    PRS 45.0%
  • transit amplifying cell CL0009010
    CSI 1.3
    rCSI 2.0%
    PRS 60.7%
  • glial cell CL0000125
    CSI 1.3
    rCSI 5.1%
    PRS 38.0%
  • glycinergic amacrine cell CL4030028
    CSI 1.4
    rCSI 3.5%
    PRS 44.2%
  • mature B cell CL0000785
    CSI 1.4
    rCSI 1.2%
    PRS 53.8%
  • epicardial adipocyte CL1000309
    CSI 1.4
    rCSI 4.5%
    PRS 46.5%
  • conjunctival epithelial cell CL1000432
    CSI 1.4
    rCSI 2.1%
    PRS 45.1%
  • cardiac neuron CL0010022
    CSI 1.4
    rCSI 4.5%
    PRS 41.4%
  • mononuclear phagocyte CL0000113
    CSI 1.4
    rCSI 3.1%
    PRS 48.3%
  • pancreatic acinar cell CL0002064
    CSI 1.4
    rCSI 1.9%
    PRS 49.2%
  • lung secretory cell CL1000272
    CSI 1.4
    rCSI 3.5%
    PRS 42.5%
  • cardiac muscle cell CL0000746
    CSI 1.4
    rCSI 2.1%
    PRS 36.1%
  • hematopoietic multipotent progenitor cell CL0000837
    CSI 1.4
    rCSI 3.4%
    PRS 62.3%
  • Bergmann glial cell CL0000644
    CSI 1.5
    rCSI 2.0%
    PRS 40.9%
  • common dendritic progenitor CL0001029
    CSI 1.5
    rCSI 1.9%
    PRS 54.0%
  • acinar cell CL0000622
    CSI 1.5
    rCSI 2.2%
    PRS 55.6%
  • pulmonary alveolar type 2 cell CL0002063
    CSI 1.5
    rCSI 2.3%
    PRS 54.4%
  • Schwann cell CL0002573
    CSI 1.5
    rCSI 4.3%
    PRS 44.4%
  • mucous neck cell CL0000651
    CSI 1.5
    rCSI 2.2%
    PRS 57.9%
  • neuroendocrine cell CL0000165
    CSI 1.5
    rCSI 5.9%
    PRS 62.6%
  • tracheobronchial smooth muscle cell CL0019019
    CSI 1.5
    rCSI 2.7%
    PRS 52.9%
  • epithelial cell of lower respiratory tract CL0002632
    CSI 1.5
    rCSI 1.2%
    PRS 44.9%
  • T-helper 17 cell CL0000899
    CSI 1.5
    rCSI 1.2%
    PRS 66.4%
  • inhibitory interneuron CL0000498
    CSI 1.5
    rCSI 3.5%
    PRS 36.5%
  • OFF-bipolar cell CL0000750
    CSI 1.5
    rCSI 2.1%
    PRS 54.3%
  • foveolar cell of stomach CL0002179
    CSI 1.5
    rCSI 3.3%
    PRS 59.4%
  • renal beta-intercalated cell CL0002201
    CSI 1.6
    rCSI 3.7%
    PRS 46.6%

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)

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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 [HIBCH](/details-gene/26275) encodes 3-hydroxyisobutyryl-CoA hydrolase, a mitochondrial enzyme that plays a critical role in the catabolism of the branched-chain amino acid valine. Functionally, it is essential for the [valine catabolic process](/details-go/GO:0006574), and its deficiency is associated with a rare autosomal recessive metabolic disorder characterized by progressive infantile neurodegeneration ([250620](https://omim.org/entry/250620)) [Link](https://doi.org/10.1086/510725). Expression data indicates that **Overall**, [HIBCH](/details-gene/26275) is highly significant in a diverse range of metabolically active cells, including epithelial cells of the lung and kidney, various neuronal subtypes, and developing thymocytes, underscoring its fundamental role in cellular energy metabolism across multiple tissues. ## Cellular Roles and Expression Landscape The expression profile of [HIBCH](/details-gene/26275) suggests its importance in fundamental cellular metabolism rather than as a marker for a specific lineage. Its highest significance is observed in cells with high energy demands or specialized metabolic functions. **Overall**, the gene shows prominent significance in epithelial tissues, as evidenced by its top ranking in [epithelial cell of lung](/details-cell/CL0000082) (CSI: 15.74) and [epithelial cell of proximal tubule](/details-cell/CL0002306) (CSI: 13.74). This is consistent with the high metabolic activity required for transport and barrier functions in these tissues. Furthermore, [HIBCH](/details-gene/26275) is highly significant in the nervous system, with strong signals in multiple neuronal populations, including [near-projecting glutamatergic cortical neuron](/details-cell/CL4023012) (CSI: 11.68) and [pvalb GABAergic cortical interneuron](/details-cell/CL4023018) (CSI: 9.93). This widespread neuronal expression aligns with the severe neurological phenotype observed in patients with mutations in this gene [Link](https://doi.org/10.1086/510725). The gene also appears to play a role in the immune system, particularly during lymphocyte development, as indicated by its high significance in [double negative thymocyte](/details-cell/CL0002489) (CSI: 12.94). Its expression in [intermediate monocyte](/details-cell/CL0002393) and [plasmablast](/details-cell/CL0000980) suggests a continuing role in the metabolic programming of mature immune cells. The broad expression across metabolically demanding cell types like [enteroendocrine cell](/details-cell/CL0000164) and [adipocyte](/details-cell/CL0000136) further reinforces its role as a key component of core cellular metabolism. ## Pathways and Molecular Function [HIBCH](/details-gene/26275) is a well-characterized enzyme with a specific metabolic function. Gene Ontology annotations confirm its localization to the [mitochondrion](/details-go/GO:0005739), specifically the [mitochondrial matrix](/details-go/GO:0005759), and its molecular function as a [3-hydroxyisobutyryl-coa hydrolase activity](/details-go/GO:0003860). The primary biological process associated with [HIBCH](/details-gene/26275) is the [branched-chain amino acid catabolic process](/details-go/GO:0009083), with a specific role in the breakdown of valine. This is further detailed by Reactome pathways, which place the gene within the [Branched-chain amino acid catabolism](/details-reactome/R-HSA-70895) pathway. The clinical significance of this function is highlighted by its direct link to the [3-hydroxyisobutyryl-coa hydrolase deficiency](/details-reactome/R-HSA-9916722) disease pathway, which is classified under [Diseases of branched-chain amino acid catabolism](/details-reactome/R-HSA-9865118). This pathway annotation is entirely consistent with its high expression in metabolically active cells that rely on diverse fuel sources, including amino acids. ## Research Directions The widespread expression of [HIBCH](/details-gene/26275) in metabolically active tissues, combined with the severe neuro-specific phenotype of its deficiency, suggests that certain cell types are uniquely vulnerable to defects in valine catabolism. This provides several avenues for future investigation. **Proposed Hypotheses:** 1. The high significance of [HIBCH](/details-gene/26275) in multiple neuronal subtypes suggests that neurons have a critical, non-redundant reliance on valine catabolism for maintaining mitochondrial health and ATP production. The accumulation of toxic intermediates, such as methacrylyl-CoA, resulting from [HIBCH](/details-gene/26275) deficiency may be particularly neurotoxic, leading to the observed progressive neurodegeneration. 2. The high expression in [epithelial cell of lung](/details-cell/CL0000082) and [epithelial cell of proximal tubule](/details-cell/CL0002306) indicates that these cells may utilize branched-chain amino acids as a significant energy source to fuel their transport and homeostatic functions. Dysfunction of [HIBCH](/details-gene/26275) could impair tissue integrity or function in these organs under conditions of metabolic stress, a phenotype that may be subclinical compared to the severe neurological effects. **Experimental Approach:** To test the first hypothesis regarding neuronal vulnerability, iPSC-derived cortical neurons could be generated from patients with known [HIBCH](/details-gene/26275) mutations and from healthy controls. These neuronal cultures could be subjected to metabolic profiling using Seahorse XF analysis to measure mitochondrial respiration and glycolysis when supplemented with different substrates, including valine. Furthermore, mass spectrometry could be used to quantify the accumulation of upstream toxic metabolites. Neuronal health could be assessed by measuring synaptic activity via multi-electrode arrays and markers of apoptosis (e.g., cleaved caspase-3) to determine if patient-derived neurons exhibit functional deficits and increased cell death, particularly when challenged with high valine loads. **Therapeutic Potential:** As [HIBCH](/details-gene/26275) deficiency is a loss-of-function metabolic disorder, therapeutic strategies would focus on restoration or compensation rather than inhibition. The enzyme's mitochondrial localization presents a challenge for traditional protein replacement therapies. Therefore, potential avenues include: * **Gene Therapy:** AAV-mediated delivery of a functional copy of the [HIBCH](/details-gene/26275) gene to the central nervous system could offer a durable treatment by restoring enzymatic activity in the most critically affected cells. * **Substrate Reduction Therapy:** Dietary management involving a low-valine or low-protein diet is a current approach to limit the accumulation of toxic upstream metabolites. * **Pharmacological Chaperones or Activators:** For patients with missense mutations that cause protein misfolding or reduced activity, small molecules could potentially be developed to stabilize the HIBCH protein or enhance its residual enzymatic function.

Genular Protein ID: 3382916657

Symbol: HIBCH_HUMAN

Name: 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 8824301

Title: Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase.

PubMed ID: 8824301

DOI: 10.1074/jbc.271.42.26430

PubMed ID: 15815621

Title: Generation and annotation of the DNA sequences of human chromosomes 2 and 4.

PubMed ID: 15815621

DOI: 10.1038/nature03466

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: 19608861

Title: Lysine acetylation targets protein complexes and co-regulates major cellular functions.

PubMed ID: 19608861

DOI: 10.1126/science.1175371

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 23186163

Title: Toward a comprehensive characterization of a human cancer cell phosphoproteome.

PubMed ID: 23186163

DOI: 10.1021/pr300630k

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

PubMed ID: 17160907

Title: Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.

PubMed ID: 17160907

DOI: 10.1086/510725

Sequence Information:

  • Length: 386
  • Mass: 43482
  • Checksum: 11B0632ED1DF2FFA
  • Sequence:
  • MGQREMWRLM SRFNAFKRTN TILHHLRMSK HTDAAEEVLL EKKGCTGVIT LNRPKFLNAL 
    TLNMIRQIYP QLKKWEQDPE TFLIIIKGAG GKAFCAGGDI RVISEAEKAK QKIAPVFFRE 
    EYMLNNAVGS CQKPYVALIH GITMGGGVGL SVHGQFRVAT EKCLFAMPET AIGLFPDVGG 
    GYFLPRLQGK LGYFLALTGF RLKGRDVYRA GIATHFVDSE KLAMLEEDLL ALKSPSKENI 
    ASVLENYHTE SKIDRDKSFI LEEHMDKINS CFSANTVEEI IENLQQDGSS FALEQLKVIN 
    KMSPTSLKIT LRQLMEGSSK TLQEVLTMEY RLSQACMRGH DFHEGVRAVL IDKDQSPKWK 
    PADLKEVTEE DLNNHFKSLG SSDLKF