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Abnormal insulin like growth factor binding protein acid labile subunit level

MedGen UID:
1390394
•Concept ID:
C4476922
•
Finding
HPO: HP:0031034

Definition

A deviation from the normal blood concentration of the insulin like growth factor binding protein acid labile subunit (IGFALS; Entrez Gene ID 3483). The acid-labile subunit (IGFALS) acts in the insulin-like growth (IGF) system by binding circulating IGF1 in a ternary complex with binding protein (IGFBP)-3 to prevent IGF1 from crossing the endothelial barrier. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal insulin like growth factor binding protein acid labile subunit level

Professional guidelines

PubMed

Noonan syndrome: relationships between genotype, growth, and growth factors.
Limal JM, Parfait B, Cabrol S, Bonnet D, Leheup B, Lyonnet S, Vidaud M, Le Bouc Y
J Clin Endocrinol Metab 2006 Jan;91(1):300-6. Epub 2005 Nov 1 doi: 10.1210/jc.2005-0983. PMID: 16263833

Recent clinical studies

Therapy

Polysomnographic sleep, growth hormone insulin-like growth factor-I axis, leptin, and weight loss.
Rasmussen MH, Wildschiødtz G, Juul A, Hilsted J
Obesity (Silver Spring) 2008 Jul;16(7):1516-21. Epub 2008 May 8 doi: 10.1038/oby.2008.249. PMID: 18464752
Idiopathic short stature: will genetics influence the choice between GH and IGF-I therapy?
Savage MO, Camacho-Hübner C, David A, Metherell LA, Hwa V, Rosenfeld RG, Clark AJ
Eur J Endocrinol 2007 Aug;157 Suppl 1:S33-7. doi: 10.1530/EJE-07-0292. PMID: 17785695
Noonan syndrome: relationships between genotype, growth, and growth factors.
Limal JM, Parfait B, Cabrol S, Bonnet D, Leheup B, Lyonnet S, Vidaud M, Le Bouc Y
J Clin Endocrinol Metab 2006 Jan;91(1):300-6. Epub 2005 Nov 1 doi: 10.1210/jc.2005-0983. PMID: 16263833
Effect of chronic renal failure and growth hormone therapy on the insulin-like growth factors and their binding proteins.
Powell DR, Liu F, Baker BK, Hinzt RL, Kale A, Suwanichkul A, Durham SK
Pediatr Nephrol 2000 Jul;14(7):579-83. doi: 10.1007/s004670000349. PMID: 10912522
Effects of renal failure on the growth hormone-insulin-like growth factor axis.
Powell DR
J Pediatr 1997 Jul;131(1 Pt 2):S13-6. doi: 10.1016/s0022-3476(97)70003-9. PMID: 9255220

Prognosis

Identified plasma proteins related to vascular structure are associated with coarctation of the aorta in children.
Ma S, Zheng J, Xu Y, Yang Z, Zhu Y, Su X, Mo X
Ital J Pediatr 2020 May 19;46(1):63. doi: 10.1186/s13052-020-00830-7. PMID: 32430056Free PMC Article
IGF-I-IGFBP-3-acid-labile subunit (ALS) complex in children and adolescents with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH).
Völkl TM, Rauh M, Schöfl C, Dörr HG
Growth Horm IGF Res 2011 Aug;21(4):191-8. Epub 2011 Jun 1 doi: 10.1016/j.ghir.2011.05.001. PMID: 21636299
Idiopathic short stature: will genetics influence the choice between GH and IGF-I therapy?
Savage MO, Camacho-Hübner C, David A, Metherell LA, Hwa V, Rosenfeld RG, Clark AJ
Eur J Endocrinol 2007 Aug;157 Suppl 1:S33-7. doi: 10.1530/EJE-07-0292. PMID: 17785695
Growth hormone secretagogues in critical illness.
Van den Berghe G
Horm Res 1999;51 Suppl 3:21-8. doi: 10.1159/000053158. PMID: 10592440
The insulin-like growth factor and binding protein axis in children with end-stage liver disease before and after orthotopic liver transplantation.
Holt RI, Baker AJ, Jones JS, Miell JP
Pediatr Transplant 1998 Feb;2(1):76-84. PMID: 10084765

Clinical prediction guides

IGF-1 Levels, Complex Formation, and IGF Bioactivity in Growth Hormone-Treated Children With Prader-Willi Syndrome.
Bakker NE, van Doorn J, Renes JS, Donker GH, Hokken-Koelega AC
J Clin Endocrinol Metab 2015 Aug;100(8):3041-9. Epub 2015 May 29 doi: 10.1210/jc.2015-1410. PMID: 26050733
IGF-I-IGFBP-3-acid-labile subunit (ALS) complex in children and adolescents with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH).
Völkl TM, Rauh M, Schöfl C, Dörr HG
Growth Horm IGF Res 2011 Aug;21(4):191-8. Epub 2011 Jun 1 doi: 10.1016/j.ghir.2011.05.001. PMID: 21636299
Human acid-labile subunit deficiency: clinical, endocrine and metabolic consequences.
Domené HM, Hwa V, Argente J, Wit JM, Camacho-Hübner C, Jasper HG, Pozo J, van Duyvenvoorde HA, Yakar S, Fofanova-Gambetti OV, Rosenfeld RG; International ALS Collaborative Group
Horm Res 2009;72(3):129-41. Epub 2009 Sep 1 doi: 10.1159/000232486. PMID: 19729943
Noonan syndrome: relationships between genotype, growth, and growth factors.
Limal JM, Parfait B, Cabrol S, Bonnet D, Leheup B, Lyonnet S, Vidaud M, Le Bouc Y
J Clin Endocrinol Metab 2006 Jan;91(1):300-6. Epub 2005 Nov 1 doi: 10.1210/jc.2005-0983. PMID: 16263833
Effects of renal failure on the growth hormone-insulin-like growth factor axis.
Powell DR
J Pediatr 1997 Jul;131(1 Pt 2):S13-6. doi: 10.1016/s0022-3476(97)70003-9. PMID: 9255220

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