Mouse, Rat, (predicted: Human, Dog, Pig, Cow, Sheep, )
产品应用
WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
分 子 量
42kDa
细胞定位
细胞核
性 状
Liquid
浓 度
1mg/ml
免 疫 原
KLH conjugated synthetic peptide derived from human LMX1b/NPS1:111-210/379
亚 型
IgG
纯化方法
affinity purified by Protein A
储 存 液
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
This gene encodes a member of LIM-homeodomain family of proteins containing two N-terminal zinc-binding LIM domains, 1 homeodomain, and a C-terminal glutamine-rich domain. It functions as a transcription factor, and is essential for the normal development of dorsal limb structures, the glomerular basement membrane, the anterior segment of the eye, and dopaminergic and serotonergic neurons. Mutations in this gene are associated with nail-patella syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010]
Function: Essential for the specification of dorsal limb fate at both the zeugopodal and autopodal levels.
Subcellular Location: Nucleus.
Tissue Specificity: Expressed in most tissues. Highest levels in testis, thyroid, duodenum, skeletal muscle, and pancreatic islets.
DISEASE: Defects in LMX1B are the cause of nail-patella syndrome (NPS) [MIM:161200]; also known as onychoosteodysplasia. NPS is a disease that cause abnormal skeletal patterning and renal dysplasia.