Overview |
bs-6643R-Biotin |
SCYL1BP1 Polyclonal Antibody, Biotin Conjugated |
WB, ELISA, IHC-P, IHC-F |
Human |
Mouse, Rat, Dog, Cow, Pig, Horse, Chicken, Rabbit |
Specifications |
Biotin |
Rabbit |
KLH conjugated synthetic peptide derived from human SCYL1BP1 |
201-300/394 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
Store at -20°C for 12 months. |
Target |
92344 |
Q5T7V8 |
Cytoplasm |
GO; NTKLBP1; SCYL1BP1; RAB6-interacting golgin; N-terminal kinase-like-binding protein 1; NTKL-BP1; NTKL-binding protein 1; hNTKL-BP1; SCY1-like 1-binding protein 1; SCYL1-BP1; SCYL1-binding protein 1; GORAB |
Defects in GORAB are the cause of geroderma osteodysplasticum (GO) [MIM:231070]; also known as gerodermia osteodysplastica or Walt Disney dwarfism. GO is a rare autosomal recessive disorder characterized by lax, wrinkled skin, joint laxity and a typical face with a prematurely aged appearance. Skeletal signs include severe osteoporosis leading to frequent fractures, malar and mandibular hypoplasia and a variable degree of growth retardation. |
Application Dilution |
WB |
1:300-5000 |
ELISA |
1:500-1000 |
IHC-P |
1:200-400 |
IHC-F |
1:100-500 |