FAM134B Rabbit pAb
FAM134B Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q9H6L5 |
| Reactivity | Rat, Human, Mouse |
| Predicted | Rabbit |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 54681 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human FAM134B |
| Epitope Specificity | 210-310/497 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Golgi apparatus; cis-Golgi network membrane. |
| DISEASE | Defects in FAM134B are the cause of hereditary sensory and autonomic neuropathy type 2B (HSAN2B) [MIM:613115]. A form of hereditary sensory and autonomic neuropathy, a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by sensory and/or autonomic abnormalities. HSAN2B is an autosomal recessive disorder characterized by impairment of pain, temperature and touch sensation. Onset occurs in the first or second decade, with impaired nociception and progressive mutilating ulceration of the hands and feet with osteomyelitis and acroosteolysis. Amputations of the hands and feet are common. Autonomic dysfunction includes hyperhidrosis, urinary incontinence, and slow pupillary light response. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Required for long-term survival of nociceptive and autonomic ganglion neurons. |
| Gene ID | 54463 |
|---|---|
| Other Names | Reticulophagy regulator 1 {ECO:0000312|HGNC:HGNC:25964}, Reticulophagy receptor 1, RETREG1 (HGNC:25964) |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=0.2ug/test |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | RETREG1 (HGNC:25964) |
|---|---|
| Function | Endoplasmic reticulum (ER)-anchored autophagy regulator which mediates ER delivery into lysosomes through sequestration into autophagosomes (PubMed:26040720, PubMed:31930741, PubMed:34338405). Promotes membrane remodeling and ER scission via its membrane bending capacity and targets the fragments into autophagosomes via interaction with ATG8 family proteins (PubMed:26040720, PubMed:31930741, PubMed:34338405). Active under basal conditions (PubMed:34338405). Required for collagen quality control in a LIR motif-dependent manner (By similarity). Required for long-term survival of nociceptive and autonomic ganglion neurons (PubMed:19838196, PubMed:26040720). |
| Cellular Location | Golgi apparatus, cis-Golgi network membrane {ECO:0000250|UniProtKB:Q8VE91}; Multi-pass membrane protein. Endoplasmic reticulum membrane; Multi-pass membrane protein |
| Tissue Location | Overexpressed in esophageal squamous cell carcinoma (PubMed:17487424). |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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