DIF14/LMBR1,分化相关基因14抗体

产品编号HZ-9563R
英文名称DIF14/LMBR1
中文名称分化相关基因14抗体
别 名ACHP; C7orf2; DIF 14; DIF14; Differentiation related gene 14; Differentiation related gene 14 protein; Differentiation-related gene 14 protein; FLJ11665; Limb region 1 homolog (mouse); Limb region 1 homolog; Limb region 1 protein; Limb region 1 protein homolog; LMBR 1; LMBR1; LMBR1_HUMAN; PPD 2; PPD2; TPT.
说 明 书0.1ml 0.2ml
研究领域细胞生物 免疫学 发育生物学 细胞分化
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
DIF14/LMBR1,分化相关基因14抗体产品应用WB=1:100-500 ELISA=1:500-1000 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55kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human LMBR1/DIF14
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Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
DIF14/LMBR1,分化相关基因14抗体PubMedPubMed
产品介绍background:
LMBR1 is a 490 amino acid multi-pass membrane protein that is widely expressed with strongest expression in heart and pancreas. Belonging to the LIMR family, LMBR1 shares 95% sequence identity with the mouse protein and may play crucial role in the evolution of limb and skeletal system. LMBR1 is critical for expression of sonic hedgehog (Shh) in the developing posterior limb bud mesenchyme. Mutations in the gene encoding LMBR1 is the cause of several rare conditions such as acheiropody (ACHP) and syndactyly type 4 (SDYT4). ACHP is an autosomal recessive inherited disorder characterized by bilateral congenital amputations of the hands and feet. LMBR1L (limb region 1 protein homolog-like), also known as LIMR (Lipocalin-1-interacting membrane receptor), is a 489 amino acid multi-pass membrane protein that is thought to act as a receptor for Lipocalin-1 and may also assist in its endocytosis.

Function:
Putative membrane receptor.

Subcellular Location:
Membrane.

Tissue Specificity:
Widely expressed with strongest expression in heart and pancreas.

DISEASE:
Defects in LMBR1 are associated with preaxial polydactyly type 2 (PPD2); also known as polydactyly of triphalangeal thumb. Polydactyly consists of duplication of the distal phalanx. The thumb in PPD2 is usually opposable and possesses a normal metacarpal. The mutations do not change the normal expression of LMBR1, but alter the expression of SHH by disrupting a long-range, cis-regulatory element of that gene.

DIF14/LMBR1,分化相关基因14抗体Similarity:
Belongs to the LIMR family.

Database links:
Entrez Gene: 64327 Human
Entrez Gene: 56873 Mouse
Entrez Gene: 362295 Rat
Omim: 605522 Human
SwissProt: Q8WVP7 Human
SwissProt: Q9JIT0 Mouse
Unigene: 209989 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.