Before biotin treatment After biotin treatment (8 mo)
Proteins Amount (μg / mg serum protein) Proteins

Amount (μg / mg serum protein)
Human serum albumin (1-) 422 Human serum albumin (1-) 227
Keratin-associated protein 4-1 (29-) 19.3 von Willebrand factor C domain-containing protein 2-like (93- 83.0
Hemopexin (Hyaluronidase; 361-) 19.3 Membrane-spanning 4-domains subfamily A member 10 (136-) 42.0
Probable E3 ubiquitin-protein ligase HERC2 (4755-) 12.5 Estrogen sulfotransferase (178-) 18.4
Leucine-rich repeat transmembrane neuronal protein 1 (424-) 11.7 Signal-induced proliferation-associated protein 1 (704-) 18.3
Alpha-2-Macroglobulin receptor-associated protein (86-)   10.7 Serum amyloid P-component (120-) 16.5
MAP kinase-activating death domain protein (373-) 10.1 Antisecretory factor 1 (120-) 14.8
Histone-lysine N-methyltransferase MLL3 (250-) 9.3 Cathepsin S (40-) 14.0
ATP-dependent helicase CHD1 (1595-) 9.1 Cell cycle control protein 50C (26-) 13.9
HBxAg transactivated protein 2 (728-) 8.6 39S Ribosomal protein L9, mitochondrial (183-) 13.5
Ceroid-lipofuscinosis neronal protein 6 (87-) 7.0 Integrin alpha-4 (55-) 11.2
RNA polymerase-associated protein CTR9 homolog (822-)    7.0 Transferrin (1-) 10.8
Embryonal Fyn-associated substrate (473-) 7.0 Mucin-6 (715-) 10.8
Zinc finger protein 441 (581-) 6.2 Zinc finger protein 800 (354-) 9.9
Nuclear autoantigenic sperm protein (710-) 6.2 Beta-Ketoacyl-ACP synthase (356-) 9.9
Importin-11 (212-) 6.1 Diphthamide biosynthesis protein 1 (141-) 9.7
Olfactory receptor 5A2 (OR; 21-) 6.0 Mannose receptor-like protein 2 (285-) 8.7
Mutated in bladder cancer protein 1 (170-) 5.4 Equilibrative nucleoside transporter 3 (224-) 6.3
Vacuolar proton pump subunit H (416-) 5.3 DNA-dependent protein kinase catalytic subunit (381-) 5.9
Potassium voltage-gated channel subfamily H member 5 (320-) 5.3 Ribonucleotide reductase small subunit (86-) 5.8
Calcium-binding mitochondrial carrier protein SCaMC-1 (427-) 4.9 Transmembrane protease, serine 9 (715-) 5.5
Sodium bicarbonate transporter-like protein 11 (118-) 4.9 Extracellular matrix protein 2 (550-) 5.4
Immunoglobulin-like and fibronectin type III domain-containing protein 1 (641-) 4.8 Lysosomal-trafficking regulator (2147-) 5.3
Transferrin (1-) 4.6 Cysteine-rich motor neuron 2 protein (151-) 5.1
Interleukin-9 receptor (295-) 4.5 N-myc downstream-regulated 1 porotein (248-) 4.7
Retinoic acid-induced protein 2 (341-) 4.4 Triple functional domain protein (288-) 4.4
Mutated in bladder cancer protein 1 (170-) 5.4 Equilibrative nucleoside transporter 3 (224-) 6.3
Serotonin receptor 4 (169-) 4.4 Alpha2-Macroglobulin (1-) 4.2
Complement factor H-related protein 3 (121-) 4.2 Renal carcinoma antigen NY-REN-36 (1254-) 4.1
Inositol monophosphatase 3 (107-) 3.9 Remained Fibrinogen (2-) 4.0
Immunogloblin superfamily member 3 (254-) 3.7 Paternally-expressed gene 3 protein (1341-) 4.0
E3 ubiquitin-protein ligase UBR3 (877-) 3.2 Alpha-fetoprotein (AFP; 1-) 3.8
Huntingtin-interacting protein HIP3RP (462-) 3.1 Receptor tyrosine-protein kinase ErbB-1 (EGF receptor; 280-) 3.6
Ceruloplasmin (19-) 0.6 Mediator complex subunit 14 (667-) 3.5
Uncharacterized protein C17orf53 (438-) 3.4 Ceruloplasmin (214-) 2.7
Alanine-glyoxylate aminotransferase  2, mitochondrial  (110-) 3.3 ATP synthase subunit gamma, mitochondrial (103-) 3.1
Ubiquitin thioesterase 27 (48-) 3.3 Biotinidase (serum sequence) (1-) 2.8
Solute carrier family 22 member 14 (446-) 3.0 Transcription initiation factor TFIID subunit 3 (515-) 2.7
Alpha2-Macroglobulin (1-) 3.0 Beta-1,4 N-acetylgalactosaminyltransferase  1 (120-) 2.7
Dyslexia susceptibility 1 candidate gene 1 protein (272-)     3.0 A-kinase anchor protein 13 (2396-) 2.7  
Biotinidase (serum sequence) (1-) 2.8 Alpha1-Antitrypsin (25-) 2.5
Patched domain-containing protein 1 (186-) 2.7 Glycosyltransferase 1 domain-containing protein 1 (196-) 2.4
C-type lectin domain family 4 member D (41-) 2.7 Patched domain-containing protein 1 (186-) 2.7
A disintegrin and metalloproteinase domain 22 (319-) 2.3 USP6 N-teminal-like protein (432-) 2.3
Bile salt export pump (737-) 2.3 Centrosomal protein of 110 kDa (545-) 2.1
Alpha-fetoprotein (AFP; 1-) 1.9 Tyrosine-protein kinase receptor ETK1 (562-) 1.6
Remained Fibrinogen (2-) 1.7 Myotubularin-related protein 8 (233-) 1.5
HBV X-associated protein 1 (618-) 1.7 Ribonuclease inhibitor (86-) 1.5
Alpha1-Antitrypsin (25-) 1.6 Beta-1,4-Galactosyltransferase  6 (12-) 1.5
Brain calcium channel II (1883-) 1.4    
Patched domain-containing protein 1 (186-) 2.7 Glycosyltransferase 1 domain-containing protein 1 (196-) 2.4
Total 701 Total 666
Invaded microbe’s protein 149 Invaded microbe’s protein 184
*Proteins coming from the cellular membranes were indicated in Italics.Glycochain synthetase were indicated as in bold. Serum of after biotin treatment is 8 mo after the biotin treatment. Transplantation operation of the liver from her father in this patient was performed after 6 mo from the initiation of the biotin treatment. IgG content was considered as 150 μg/mg serum protein. Membrane proteins present in the serum were estimated as 84 and 32 μg/mg serum protein for before and after biotin treatment, respectively. Proteins of invaded microbes were shown in the Supplementary data 3 (excel tables).Other conditions were as described in the Materials and Methods section.
Table 1: Component serum proteins of Child patient 1 with GSD1b before and after the biotin administration.