Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines

Figures & data

Table 1. Amyloid fibril proteins and their precursors in human^a.

Fibril protein	Precursor protein	Systemic and/ or localized	Acquired or hereditary	Target organs
AL	Immunoglobulin light chain	S, L	A, H	All organs, usually except CNS
AH	Immunoglobulin heavy chain	S, L	A	All organs except CNS
AA	(Apo) Serum amyloid A	S	A	All organs except CNS
ATTR	Transthyretin, wild type	S	A	Heart mainly in males, ligaments, tenosynovium
	Transthyretin, variants	S	H	PNS, ANS, heart, eye, leptomeninges
Aβ2M	β2-Microglobulin, wild type	S	A	Musculoskeletal system
	β2-Microglobulin, variant	S	H	ANS
AApoAI	Apolipoprotein A I, variants	S	H	Heart, liver, kidney, PNS, testis, larynx (C-terminal variants), skin (C-terminal variants)
AApoAII	Apolipoprotein A II, variants	S	H	Kidney
AApoAIV	Apolipoprotein A IV, wild type	S	A	Kidney medulla and systemic
AApoCII	Apolipoprotein C II, variants	S	H	Kidney
AApoCIII	Apolipoprotein C III, variants	S	H	Kidney
AGel	Gelsolin, variants	S	H	PNS, cornea
ALys	Lysozyme, variants	S	H	Kidney
ALECT2	Leukocyte chemotactic factor-2	S	A	Kidney, primarily
AFib	Fibrinogen α, variants	S	H	Kidney, primarily
ACys	Cystatin C, variants	S	H	PNS, skin
ABri	ABriPP, variants	S	H	CNS
ADan*	ADanPP, variants	L	H	CNS
Aβ	Aβ protein precursor, wild type	L	A	CNS
	Aβ protein precursor, variant	L	H	CNS
AαSyn	α-Synuclein	L	A	CNS
ATau	Tau	L	A	CNS
APrP	Prion protein, wild type	L	A	CJD, fatal insomnia
	Prion protein variants	L	H	CJD, GSS syndrome, fatal insomnia
	Prion protein variant	S	H	PNS
ACal	(Pro)calcitonin	L	A	C-cell thyroid tumors
AIAPP	Islet amyloid polypeptide**	L	A	Islets of Langerhans, insulinomas
AANF	Atrial natriuretic factor	L	A	Cardiac atria
APro	Prolactin	L	A	Pituitary prolactinomas, aging pituitary
AIns	Insulin	L	A	Iatrogenic, local injection
ASPC***	Lung surfactant protein	L	A	Lung
AGal7	Galectin 7	L	A	Skin
ACor	Corneodesmosin	L	A	Cornified epithelia, hair follicles
AMed	Lactadherin	L	A	Senile aortic, media
AKer	Kerato-epithelin	L	A	Cornea, hereditary
ALac	Lactoferrin	L	A	Cornea
AOAAP	Odontogenic ameloblast-associated protein	L	A	Odontogenic tumors
ASem1	Semenogelin 1	L	A	Vesicula seminalis
AEnf	Enfurvitide	L	A	Iatrogenic

^aProteins are listed, when possible, according to relationship. Thus, apolipoproteins are grouped together, as are polypeptide hormones.

* ADan is the product of the same gene as ABri.

** Also called amylin.

*** Not proven by amino acid sequence analysis.

Table 2. Amyloid fibril proteins and their precursors in animals.

Fibril protein	Precursor protein	Systemic and/ or localized	Affected organs or syndrome	Species
AL	Immunoglobulin light chain	S, L	Plasmacytoma	Cat, Horse
AA	(Apo) Serum amyloid A	S	Chronic inflammation or infections	Mouse, Cat, Cow, Dog, Duck, Guinea pig, etc.
AApoAI	Apolipoprotein AI	S	Age-related	Dog
AApoAII	Apolipoprotein AII	S	Age-related	Mouse
ATTR	Transthyretin, variant	S	Age-related	Vervet monkey
AFib	Fibrinogen Aα	S	Spleen, liver	Stone marten
Aβ	Aβ precursor protein	L	Age-related	Dog, Sheep, Wolverine
AIAPP	Islet amyloid polypeptide	L	Islets of Langerhans, insulinoma	Apes, Cat, Racoon
AIns	Insulin	L	Islets of Langerhans	Octodon degus
ACas	A-S2C casein	L	Mammary gland	Cow

Table 3. Intracellular inclusions with known biochemical composition, with or without amyloid properties.

Inclusion name	Site	Protein nature	Examples of associated disease
Lewy bodies	Neurons intracytoplasmic	α-synuclein*,**	Parkinson’s disease
Huntington bodies	Neurons intranuclear	PolyQ expanded huntingtin	Huntington’s disease
Hirano bodies	Neurons	Actin	Neurodegenerative disorders
Collins bodies	Neurons	Neuroserpin	Forms of familial presenile dementia
Not specified	Neurons, many different cells	Ferritin	Form of familial neurodegenerative disorder
Neurofibrillary tangles	Neurons intracytoplasmic	Tau**	Alzheimer’s disease, fronto-temporal dementia, aging, other cerebral conditions
AαSyn	Neurons intracytoplasmic	α-synuclein**	Parkinson’s disease, other cerebral conditions

*Simplified. Additional components may exist.

** Also included in Table 1 since deposits may appear extracellularly.