A Concise Review of Amyloidosis in Animals

Table 1

Nomenclature and classification of amyloid and amyloidosis. Source: WHO/IUIS Nomenclature subcommittee [2].

Amyloid protein^a, b	Protein precursor	Protein type	Clinical diagnosis
AA	apoSAA		Reactive (secondary) amyloidosis, familial mediterranean fever, familial amyloid nephropathy with urticarial and deafness (Muckie-Wells’ syndrome)
AL	κλ, for example, κIII	Aκ, Aλ, for example, AκIII	Idiopathic (primary) amyloidosis associated with myeloma/macroglobulinaemia
AH	IgG1 (γ ^1)	Aγ ^1
ATTR	Transthyretin	For example, Met 30^c For example, Met III TTR or IIe 122	Familial amyloid polyneuropathy, Portuguese; Familial amyloid cardiomyopathy, Danish; Systemic senile amyloidosis
AApoAI	apoAI	Arg 26	Familial amyloid polyneuropathy, Iowa
AGel	Gelsolin	Asn 187^d (15)	Familial amyloidosis, Finish
ACys	Cystatin C	Gin 68	Hereditary cerebral hemorrhage with amyloidosis, Icelandic
Aβ	β protein precursor for example, βPP 695^e	Gin 618 (22)	Alzheimer’s disease, Down syndrome, and hereditary cerebral hemorrhage with amyloidosis, Dutch
Aβ 2M	β2-microglobulin		Associated with chronic dialysis
AScr	Scrapie protein precursor 33–35^f cellular form	Scrapie protein 27–30	Creutzfeldt-Jakob disease, and so forth
		For example, Leu 102	Gerstmann-Straüssler-Scheinker syndrome
ACal	(Pro)calcitonin	(Pro)calcitonin	In medullary carcinomas of the thyroid
AANF	Atrial natriuretic factor		Isolated atrial amyloid
AIAPP	Islet amyloid polypeptide		In islets of Langerhans, diabetes type II, insulinoma
AIns^g	Insulin		Islet amyloid in the degu (a rodent)
AApoAII^g	apoAII (murine)	Gin5	Amyloidosis in senescence, accelerated mice

^aNonfibrillar proteins, for example, protein AP (amyloid P-component) excluded.
^bAA: amyloid A protein; SAA: serum amyloid A protein; apo: apolipoprotein; L: immunoglobulin light chain; H: immunoglobulin heavy chain.
^cATTR Met 30 when used in text.
^dAmino acid position in the mature precursor protein. The position in the amyloid fibril protein is given in parentheses.
^eNumber of amino acid residues; ^fMolecular mass (kilodaltons); ^gNot found in humans.