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Rabbit Anti-GDAP1 antibody
Rabbit Anti-GDAP1 antibody
Ganglioside induced differentiation associated protein 1; Ganglioside-induced differentiation-associated protein 1; GDAP1; GDAP1_HUMAN.
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  • NO.:SL11725R
    Clonality:Polyclonal
    Immunogen Species:Rabbit
    React Species:Mouse,(predicted: Human,Rat,Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep,)
    Applications:WB ELISA IHC-P IHC-F ICC IF
    concentration:1mg/ml
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Details

Product Name GDAP1
Chinese Name 神经节苷脂诱导分化相关蛋白1抗体
Alias Ganglioside induced differentiation associated protein 1; Ganglioside-induced differentiation-associated protein 1; GDAP1; GDAP1_HUMAN.  
Research Area Cell biology  Neurobiology  
Immunogen Species Rabbit
Clonality Polyclonal
React Species Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
Applications 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 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight 41kDa
Cellular localization cytoplasmic The cell membrane 
Form Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human GDAP1: 151-230/358 
Lsotype IgG
Purification affinity purified by Protein A
Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
Product Detail Glutathione S-transferases (GSTs) function to conjugate reduced glutathione to many exogenous and endogenous hydrophobic electrophiles. Although it shares the carboxy and amino-terminal glutathione S-transferase domains, GDAP1 is characterized as a GST-like protein because it contains an extended GST domain II and a predicted transmembrane domain, two characteristics which are unusual for GST family members. GDAP1 may function in a signal transduction pathway that is responsible for ganglioside-induced neurite differentiation and also may play a role in protecting myelin membranes from free-radical damage. Mutations in the gene encoding GDAP1 is the cause of many forms of Charcot-Marie-Tooth disease, a common inherited disorder of the peripheral nervous system that is characterized by reduced nerve conduction velocities, slow progressive distal muscle atrophy and absent deep tendon reflexes.

Function:
May function in a signal transduction pathway responsible for ganglioside-induced neurite differentiation. May also have a role in protecting myelin membranes against free radical-mediated damage.

Subunit:
Homodimer.

Subcellular Location:
Mitochondrion outer membrane; Multi-pass membrane protein. Cytoplasm (By similarity).

Tissue Specificity:
Highly expressed in whole brain and spinal cord. Predominant expression in central tissues of the nervous system not only in neurons but also in Schwann cells.

DISEASE:
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease type 4A (CMT4A) [MIM:214400]. CMT4A is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Demyelinating CMT neuropathies are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. Autosomal recessive forms of demyelinating Charcot-Marie-Tooth disease are by convention designated CMT4. CMT4A is a severe form characterized by early age of onset and rapid progression leading to inability to walk in late childhood or adolescence.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease axonal recessive with vocal cord paresis (CMT2RV) [MIM:607706]. CMT2RV is a form of Charcot-Marie-Tooth disease characterized by the association of axonal neuropathy with vocal cord paresis.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease type 2K (CMT2K) [MIM:607831]. CMT2K is an axonal form of Charcot-Marie-Tooth disease. Axonal CMT neuropathies are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2K onset is in early childhood (younger than 3 years). This phenotype is characterized by foot deformities, kyphoscoliosis, distal limb muscle weakness and atrophy, areflexia, and diminished sensation in the lower limbs. Weakness in the upper limbs is observed in the first decade, with clawing of the fingers. Inheritance can be autosomal dominant or recessive.
Defects in GDAP1 are the cause of Charcot-Marie-Tooth disease recessive intermediate type A (CMTRIA) [MIM:608340]. CMTRIA is a form of Charcot-Marie-Tooth disease characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec.

Similarity:
Belongs to the GST superfamily.
Contains 1 GST C-terminal domain.
Contains 1 GST N-terminal domain.

SWISS:
Q8TB36

Gene ID:
54332

Database links:

Entrez Gene: 613472 Cow

Entrez Gene: 54332 Human

Entrez Gene: 14545 Mouse

Entrez Gene: 312890 Rat

Omim: 606598 Human

SwissProt: A6QQZ0 Cow

SwissProt: Q8TB36 Human

SwissProt: O88741 Mouse

Unigene: 168950 Human

Unigene: 18218 Mouse



Product Picture
Sample:
Large intestine (Mouse) Lysate at 40 ug
Primary: Anti- GDAP1 (SL11725R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 41 kD
Observed band size: 42 kD

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